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Jung JM, Moon IJ, Lee WJ, Won CH, Chang SE, Lee MW. Clinically assessed mycosis fungoides tumor burden index as a prognostic marker in tumor-stage mycosis fungoides: a retrospective cohort study. Arch Dermatol Res 2024; 317:42. [PMID: 39576358 DOI: 10.1007/s00403-024-03496-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2024] [Revised: 09/10/2024] [Accepted: 10/22/2024] [Indexed: 11/24/2024]
Abstract
Prognostic markers are needed for tumor-stage mycosis fungoides (MF) because of their variable prognosis. The objectives of this study were to explore prognostic markers for tumor-stage MF and assess the prognostic significance of clinically assessed MF tumor burden index (MTBI). MTBI was devised to consider the tumor size ≥ 2 cm, number ≥ 5, ulcers, and body surface area ≥ 50%. The prognostic value of MTBI and other potential markers derived from blood tests and skin biopsy were evaluated retrospectively using a tertiary medical center database. We included 38 cases of tumor-stage MF. The mean age was 52.1 years, and the male-to-female ratio was 2.5:1. In multivariable analysis, MTBI ≥ 3 (adjusted hazard ratio, 9.41; 95% confidence interval, 1.13-78.15) was significantly associated with worse disease-specific survival. Ulcers were the only MTBI constituent significantly associated with survival. Among other markers, elevated lactate dehydrogenase level was associated with a worse disease-specific survival. Neutrophil-lymphocyte-ratio, pan-inflammation-value, CD30 positivity, Ki-67 index, large cell transformation, and monoclonal T-cell receptor gene rearrangement were not associated with prognosis. In conclusion, MTBI is useful and promising prognostic marker for tumor-stage MF.
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Affiliation(s)
- Joon Min Jung
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea
| | - Ik Jun Moon
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea
| | - Woo Jin Lee
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea
| | - Chong Hyun Won
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea
| | - Sung Eun Chang
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea
| | - Mi Woo Lee
- Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Seoul, Songpa-gu, Korea.
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Hodak E, Geskin L, Guenova E, Ortiz-Romero PL, Willemze R, Zheng J, Cowan R, Foss F, Mangas C, Querfeld C. Real-Life Barriers to Diagnosis of Early Mycosis Fungoides: An International Expert Panel Discussion. Am J Clin Dermatol 2023; 24:5-14. [PMID: 36399227 PMCID: PMC9673193 DOI: 10.1007/s40257-022-00732-w] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/19/2022] [Indexed: 11/19/2022]
Abstract
Mycosis fungoides (MF) is a rare, primary cutaneous T-cell lymphoma that is challenging to diagnose due to its heterogeneous clinical presentation and complex histology. The subtlety of the initial clinical appearance of MF can result in diagnostic delays and hesitancy to refer suspected cases to specialist clinics. An unmet need remains for greater awareness and education. Therefore, an international expert panel of dermatologists, oncologists, hematologists, and dermatopathologists convened to discuss and identify barriers to early and accurate MF diagnosis that could guide clinicians toward making a correct diagnosis. Confirmation of MF requires accurate assessment of symptoms and clinical signs, and subsequent correlation with dermatopathological findings. This review summarizes the expert panel's guidance, based on the literature and real-life experience, for dermatologists to help include MF in their list of differential diagnoses, along with simple clinical and histopathologic checklists that may help clinicians to suspect and identify potential MF lesions and reduce diagnostic delays.
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Affiliation(s)
- Emmilia Hodak
- Division of Dermatology, Rabin Medical Center, Beilinson Hospital, Tel Aviv University, 39 Jabotinsky Street, Petah Tiqva, 49100, Tel Aviv, Israel.
| | - Larisa Geskin
- Columbia University Medical Center, Columbia University, New York, NY, USA
| | - Emmanuella Guenova
- University Hospital Lausanne (CHUV) and Faculty of Biology and Medicine, University of Lausanne, Lausanne, Switzerland
| | - Pablo L Ortiz-Romero
- Department of Dermatology, Hospital 12 de Octubre, Institute i+12, CIBERONC, Medical School, University Complutense, Madrid, Spain
| | - Rein Willemze
- Leiden University Medical Center, Leiden, The Netherlands
| | - Jie Zheng
- Ruijin Hospital, Shanghai Jiaotong University, Shanghai, China
| | - Richard Cowan
- Christie Hospital, The Christie School of Oncology, Manchester, UK
| | - Francine Foss
- Yale Cancer Center, Yale School of Medicine, New Haven, CT, USA
| | - Cristina Mangas
- Dermatology Department and Institute of Oncology of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland
| | - Christiane Querfeld
- Division of Dermatology and Department of Pathology, City of Hope National Medical Center and Beckman Research Institute, Duarte, CA, USA
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Pediatric Mycosis Fungoides: Retrospective Analysis of a Series With CD8 + Profile and Female Predominance. J Pediatr Hematol Oncol 2022; 44:e994-e998. [PMID: 34699461 DOI: 10.1097/mph.0000000000002354] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2021] [Accepted: 09/21/2021] [Indexed: 11/26/2022]
Abstract
BACKGROUND Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional data to better understand the clinicopathologic features of MF in children. MATERIALS AND METHODS This study was a retrospective analysis of pediatric MF patients (diagnosed at age 0 to 18 y). RESULTS Thirteen pediatric patients with MF were identified. Female predominance was observed with a ratio of 1.6:1. Median values for age of onset of skin lesions and age at the time of histologic diagnosis were 5 and 12 years, respectively. All patients had early stage (stage IA to IIA) of MF at the time of diagnosis. Hypopigmented MF comprised 77% of all study patients, followed by classic MF (15%) and pagetoid reticulosis (8%). The lower extremity (especially proximal leg) followed by trunk and upper extremity were most commonly affected sites. Seven of 9 patients who had available immunohistochemistry data showed CD8 + predominance. Five of 8 patients whose follow-up data was available, achieved complete response with narrowband ultraviolet B treatment, while 2 and 1 had near complete response and partial response, respectively. CONCLUSIONS Our study demonstrated female sex and CD8 + profile predominance. Hypopigmented MF constituted the majority of cases. We observed good responses with narrowband ultraviolet B treatment.
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Liu F, Li X, Ma JC, Wang L, Shi Z. Poikilodermatous and Ichthyosiform Patches all over the Body and Dark-brown Nodules on the Legs: A Quiz. Acta Derm Venereol 2022; 102:adv00759. [DOI: 10.2340/actadv.v102.2504] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Abstract is missing (Quiz)
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Ding X, Chen J, Kuai L, Xing M, Ru Y, Luo Y, Luo Y, Zhou M, Li B, Li X. CD4/CD8 dual-positive mycosis fungoides: A case report and literature review. Medicine (Baltimore) 2020; 99:e22786. [PMID: 33080750 PMCID: PMC7571916 DOI: 10.1097/md.0000000000022786] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
RATIONALE Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. It appears as patches, plaques, and tumors depending on the stage of the disease, which presents a chronic progressive course. Compared to CD4/CD8 MF, CD4/CD8 dual-positive MF is an uncommon immune phenotype. PATIENT CONCERNS A 36-year-old male patient presented with dryness and scales on his whole body. DIAGNOSIS The patient was diagnosed with MF based on results of pathological examination, immunohistochemical staining, and T-cell receptor gene rearrangement test. INTERVENTIONS The patient was advised to take an herbal medicine orally twice daily and apply a topical moisturizer after showering. OUTCOMES After treatment and follow-up, the patient's symptoms of dryness and scales improved and his condition stabilized. CONCLUSIONS While reviewing the literature, we found no previous reports on the treatment of dual-positive MF with Chinese medicine. In this report, we presented the first case of dual-positive MF successfully treated with Chinese medicine. The results suggest that oral ingestion of herbal medicine may be a feasible method for alleviating clinical symptoms of early stage MF. Therefore, the therapy should be explored for clinical use in the future.
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Affiliation(s)
- Xiaojie Ding
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
| | - Jia Chen
- Department of Dermatopathology, Shanghai Dermatology Hospital, Tongji University
| | - Le Kuai
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
| | - Meng Xing
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
| | - Yi Ru
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
- Institute of Dermatology, Shanghai Academy of Traditional Chinese Medicine, Shanghai, China
| | - Ying Luo
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
- Institute of Dermatology, Shanghai Academy of Traditional Chinese Medicine, Shanghai, China
| | - Yue Luo
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
| | - Mi Zhou
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
| | - Bin Li
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
- Institute of Dermatology, Shanghai Academy of Traditional Chinese Medicine, Shanghai, China
| | - Xin Li
- Department of Dermatology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine
- Institute of Dermatology, Shanghai Academy of Traditional Chinese Medicine, Shanghai, China
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Park MY, Hwang S, Kim J, Almurayshid AI, Yoon SO, Oh SH. Rare case of CD8+ CD56+ cytotoxic variant of mycosis fungoides clinically presenting with a combination of hypopigmentation and poikiloderma. Int J Dermatol 2020; 59:e374-e376. [PMID: 32627839 DOI: 10.1111/ijd.15045] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2019] [Revised: 05/31/2020] [Accepted: 06/10/2020] [Indexed: 11/30/2022]
Affiliation(s)
- Min-Young Park
- Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea
| | - Shinwon Hwang
- Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea
| | - Jemin Kim
- Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea
| | - Abdurrahman I Almurayshid
- Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.,Department of Dermatology, College of Medicine, Prince Sattam Bin Abdulaziz University, Alkharj, Saudi Arabia
| | - Sun Och Yoon
- Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Sang Ho Oh
- Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea
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Rohmer E, Mitcov M, Cribier B, Lipsker D, Lenormand C. [Clinical heterogeneity of poikilodermatous mycosis fungoides: A retrospective study of 12 cases]. Ann Dermatol Venereol 2020; 147:418-428. [PMID: 32229035 DOI: 10.1016/j.annder.2020.02.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2019] [Revised: 01/08/2020] [Accepted: 02/05/2020] [Indexed: 11/28/2022]
Abstract
INTRODUCTION Poikilodermatous mycosis fungoides is a rare and indolent clinical variant of mycosis fungoides (MF). It can be difficult to distinguish from poikilodermatous parapsoriasis, a group of chronical dermatoses that may sometimes progress to MF. We aimed to specify the clinical, histopathological and developmental features of these entities by means of a retrospective study of 12 cases followed in our center. PATIENTS AND METHODS We identified cases of poikiloderma for which a diagnosis of MF or parapsoriasis was made by the physician. Photographs and histological slides were reviewed, and a final diagnosis of MF was made if the International Society for Cutaneous Lymphoma criteria for the diagnosis of early MF were fulfilled. RESULTS Twelve patients were included, 10 of whom met of the MF criteria. 5 patients had large poikilodermatous patches or thin, well-defined plaques ; 3 patients had the same lesions associated with classical MF lesions ; finally, 4 patients had widespread ill-defined erythematous lesions in a net-like pattern, described as parakeratosis variegata, including 3 MF. 2 patients with well-defined lesions (one associated with classical MF lesions) progressed to the tumoral stage whereas none of the patients with parakeratosis variegata presented such progression. A total of 5 patients had a high skin phototype (IV and V). Two patients had squamous cell carcinoma on poikilodermatous lesions. DISCUSSION Our study suggests that poikilodermatous MF covers a heterogeneous clinical spectrum comprising on one hand a presentation of delimited lesions sharing classical MF risk of progression, and on the other, an entity similar to parakeratosis variegata, an entity overlooked in the French nomenclature, which was particularly benign in our small series, raising the question of its affiliation to the MF group. This question merits further investigation in a larger-scale study.
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Affiliation(s)
- E Rohmer
- Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
| | - M Mitcov
- Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France
| | - B Cribier
- Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France
| | - D Lipsker
- Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France
| | - C Lenormand
- Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France
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Sidiropoulou P, Nikolaou V, Marinos L, Voudouri D, Komini E, Economidi A, Rigopoulos D, Stratigos A. The different faces of mycosis fungoides: results of a single‐center study. Int J Dermatol 2019; 59:314-320. [DOI: 10.1111/ijd.14735] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2019] [Revised: 10/21/2019] [Accepted: 11/05/2019] [Indexed: 12/13/2022]
Affiliation(s)
- Polytimi Sidiropoulou
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Vasiliki Nikolaou
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Leonidas Marinos
- Hematopathology Department “Evangelismos” General Hospital Athens Greece
| | - Dimitra Voudouri
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Elena Komini
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Afroditi Economidi
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Dimitris Rigopoulos
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
| | - Alexander Stratigos
- 1st Department of Dermatology‐Venereology Faculty of Medicine National and Kapodistrian University of Athens Cutaneous Lymphoma Clinic, “A. Sygros” Hospital for Skin & Venereal Diseases Athens Greece
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9
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Kalay Yildizhan I, Sanli H, Akay BN, Sürgün E, Heper A. CD8 + cytotoxic mycosis fungoides: a retrospective analysis of clinical features and follow-up results of 29 patients. Int J Dermatol 2019; 59:127-133. [PMID: 31633200 DOI: 10.1111/ijd.14689] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2019] [Revised: 09/14/2019] [Accepted: 09/20/2019] [Indexed: 11/26/2022]
Abstract
BACKGROUND AND OBJECTIVE Less than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype. This study aimed to evaluate the clinical characteristics, treatment modalities, and clinical course in CD8+ MF patients. METHODS In a retrospective analysis of 353 MF patients in a referral center at Ankara University, Turkey, 29 patients that were diagnosed with CD8+ MF were included in the study. RESULTS CD8+ MF cases constituted 8.2% of all MF patients. The age at the time of diagnosis ranged between 6 and 81 years with a median value of 46 years. The female-to-male ratio was 1.41. Patients presented with erythematous scaly (69%), hyperpigmented (58.6%), poikilodermic (17.2%), and hypopigmented (17.2 %) patches/plaques. The most common sites of involvement were the trunk and lower extremities. The most common comorbidity was hypertension (24.1%, n: 7) with 13 patients (44.8%) having a history of at least one autoimmune disease. At the time of diagnosis, 93.2% of the patients had early-stage disease, and 6.8% of the patients had advanced stage. The mean follow-up period was 6.68 ± 6.04 years (range 1-28 years). Most of the patients were treated with skin-directed therapies. Complete remission was achieved in 17 (58.6%) patients, eight (27.6%) patients had partial remission, and four (13.8%) patients had stable disease. CONCLUSIONS We concluded that CD8+ MF is associated with an indolent course and in most patients, skin-directed therapies were found to be efficient to control the disease.
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Affiliation(s)
| | - Hatice Sanli
- Department of Dermatology, Faculty of Medicine, Ankara University, Ankara, Turkey
| | - Bengu N Akay
- Department of Dermatology, Faculty of Medicine, Ankara University, Ankara, Turkey
| | - Ece Sürgün
- Department of Dermatology, Faculty of Medicine, Ankara University, Ankara, Turkey
| | - Aylin Heper
- Department of Pathology, Faculty of Medicine, Ankara University, Ankara, Turkey
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10
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Scarisbrick JJ, Quaglino P, Prince HM, Papadavid E, Hodak E, Bagot M, Servitje O, Berti E, Ortiz-Romero P, Stadler R, Patsatsi A, Knobler R, Guenova E, Child F, Whittaker S, Nikolaou V, Tomasini C, Amitay I, Prag Naveh H, Ram-Wolff C, Battistella M, Alberti-Violetti S, Stranzenbach R, Gargallo V, Muniesa C, Koletsa T, Jonak C, Porkert S, Mitteldorf C, Estrach T, Combalia A, Marschalko M, Csomor J, Szepesi A, Cozzio A, Dummer R, Pimpinelli N, Grandi V, Beylot-Barry M, Pham-Ledard A, Wobser M, Geissinger E, Wehkamp U, Weichenthal M, Cowan R, Parry E, Harris J, Wachsmuth R, Turner D, Bates A, Healy E, Trautinger F, Latzka J, Yoo J, Vydianath B, Amel-Kashipaz R, Marinos L, Oikonomidi A, Stratigos A, Vignon-Pennamen MD, Battistella M, Climent F, Gonzalez-Barca E, Georgiou E, Senetta R, Zinzani P, Vakeva L, Ranki A, Busschots AM, Hauben E, Bervoets A, Woei-A-Jin FJSH, Matin R, Collins G, Weatherhead S, Frew J, Bayne M, Dunnill G, McKay P, Arumainathan A, Azurdia R, Benstead K, Twigger R, Rieger K, Brown R, Sanches JA, Miyashiro D, Akilov O, McCann S, Sahi H, Damasco FM, Querfeld C, Folkes A, Bur C, Klemke CD, Enz P, Pujol R, Quint K, Geskin L, Hong E, Evison F, Vermeer M, Cerroni L, Kempf W, Kim Y, Willemze R. The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients. Br J Dermatol 2019; 181:350-357. [PMID: 30267549 DOI: 10.1111/bjd.17258] [Citation(s) in RCA: 131] [Impact Index Per Article: 21.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/25/2018] [Indexed: 12/16/2022]
Abstract
BACKGROUND Survival in mycosis fungoides (MF) is varied and may be poor. The PROCLIPI (PROspective Cutaneous Lymphoma International Prognostic Index) study is a web-based data collection system for early-stage MF with legal data-sharing agreements permitting international collaboration in a rare cancer with complex pathology. Clinicopathological data must be 100% complete and in-built intelligence in the database system ensures accurate staging. OBJECTIVES To develop a prognostic index for MF. METHODS Predefined datasets for clinical, haematological, radiological, immunohistochemical, genotypic, treatment and quality of life are collected at first diagnosis of MF and annually to test against survival. Biobanked tissue samples are recorded within a Federated Biobank for translational studies. RESULTS In total, 430 patients were enrolled from 29 centres in 15 countries spanning five continents. Altogether, 348 were confirmed as having early-stage MF at central review. The majority had classical MF (81·6%) with a CD4 phenotype (88·2%). Folliculotropic MF was diagnosed in 17·8%. Most presented with stage I (IA: 49·4%; IB: 42·8%), but 7·8% presented with enlarged lymph nodes (stage IIA). A diagnostic delay between first symptom development and initial diagnosis was frequent [85·6%; median delay 36 months (interquartile range 12-90)]. This highlights the difficulties in accurate diagnosis, which includes lack of a singular diagnostic test for MF. CONCLUSIONS This confirmed early-stage MF cohort is being followed-up to identify prognostic factors, which may allow better management and improve survival by identifying patients at risk of disease progression. This study design is a useful model for collaboration in other rare diseases, especially where pathological diagnosis can be complex.
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Affiliation(s)
- J J Scarisbrick
- European Co-ordinating PROCLIPI Centre for PROCLIPI, University Hospitals Birmingham, Birmingham, U.K
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
- Member of the UK Cutaneous Lymphoma Group
| | - P Quaglino
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - H M Prince
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - E Papadavid
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - E Hodak
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - M Bagot
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - O Servitje
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - E Berti
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - P Ortiz-Romero
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Stadler
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - A Patsatsi
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Knobler
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - E Guenova
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - F Child
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the UK Cutaneous Lymphoma Group
| | - S Whittaker
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
- Member of the UK Cutaneous Lymphoma Group
| | - V Nikolaou
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - C Tomasini
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - I Amitay
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - H Prag Naveh
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - C Ram-Wolff
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M Battistella
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - S Alberti-Violetti
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - R Stranzenbach
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - V Gargallo
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - C Muniesa
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - T Koletsa
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - C Jonak
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - S Porkert
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - C Mitteldorf
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - T Estrach
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Combalia
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M Marschalko
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - J Csomor
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Szepesi
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Cozzio
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Dummer
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - N Pimpinelli
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - V Grandi
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M Beylot-Barry
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Pham-Ledard
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M Wobser
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - E Geissinger
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - U Wehkamp
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - M Weichenthal
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - R Cowan
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the UK Cutaneous Lymphoma Group
| | - E Parry
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the UK Cutaneous Lymphoma Group
| | - J Harris
- Member of the UK Cutaneous Lymphoma Group
| | - R Wachsmuth
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the UK Cutaneous Lymphoma Group
| | - D Turner
- Member of the UK Cutaneous Lymphoma Group
| | - A Bates
- Member of the UK Cutaneous Lymphoma Group
| | - E Healy
- Member of the UK Cutaneous Lymphoma Group
| | - F Trautinger
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - J Latzka
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - J Yoo
- European Co-ordinating PROCLIPI Centre for PROCLIPI, University Hospitals Birmingham, Birmingham, U.K
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - B Vydianath
- European Co-ordinating PROCLIPI Centre for PROCLIPI, University Hospitals Birmingham, Birmingham, U.K
| | - R Amel-Kashipaz
- European Co-ordinating PROCLIPI Centre for PROCLIPI, University Hospitals Birmingham, Birmingham, U.K
| | - L Marinos
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Oikonomidi
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Stratigos
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M-D Vignon-Pennamen
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - M Battistella
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - F Climent
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - E Gonzalez-Barca
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - E Georgiou
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - R Senetta
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - P Zinzani
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - L Vakeva
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Ranki
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A-M Busschots
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - E Hauben
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - A Bervoets
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - F J S H Woei-A-Jin
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - R Matin
- Member of the UK Cutaneous Lymphoma Group
| | - G Collins
- Member of the UK Cutaneous Lymphoma Group
| | | | - J Frew
- Member of the UK Cutaneous Lymphoma Group
| | - M Bayne
- Member of the UK Cutaneous Lymphoma Group
| | - G Dunnill
- Member of the UK Cutaneous Lymphoma Group
| | - P McKay
- Member of the UK Cutaneous Lymphoma Group
| | | | - R Azurdia
- Member of the UK Cutaneous Lymphoma Group
| | - K Benstead
- Member of the UK Cutaneous Lymphoma Group
| | - R Twigger
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - K Rieger
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Brown
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - J A Sanches
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - D Miyashiro
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - O Akilov
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - S McCann
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - H Sahi
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - F M Damasco
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - C Querfeld
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - A Folkes
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - C Bur
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - C-D Klemke
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - P Enz
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Pujol
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - K Quint
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - L Geskin
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - E Hong
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - F Evison
- European Co-ordinating PROCLIPI Centre for PROCLIPI, University Hospitals Birmingham, Birmingham, U.K
| | - M Vermeer
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - L Cerroni
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - W Kempf
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
| | - Y Kim
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
| | - R Willemze
- Member of the European Organisation of Research and Treatment of Cancer (EORTC), Cutaneous Lymphoma Task Force
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11
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Geller S, Hollmann TJ, Horwitz SM, Myskowski PL, Pulitzer M. C-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment. Histopathology 2019; 76:222-232. [PMID: 31355940 DOI: 10.1111/his.13960] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2019] [Accepted: 07/25/2019] [Indexed: 01/30/2023]
Abstract
AIMS Patients with aggressive CD8+ cutaneous T-cell lymphomas (CTCLs) progress rapidly and respond poorly to therapy. Confounding treatment planning, there is clinicopathological overlap between aggressive CD8+ CTCLs and other lymphoproliferative disorders (LPDs). Hence, improved diagnostic methods and therapeutic options are needed. The aim of this study was to examine C-C chemokine receptor 4 (CCR4) expression as a diagnostic and therapeutic biomarker in CD8+ CTCLs/LPDs. METHODS AND RESULTS Forty-nine cases (41 patients) with CD8+ CTCLs/LPDs were examined, including CD8+ mycosis fungoides (MF) (n = 14), aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (AETCL) (n = 8), subcutaneous panniculitis-like T-cell lymphoma (SPTCL) (n = 7), CD30+ LPDs (n = 6), primary cutaneous γδ T-cell lymphoma (GDTCL) (n = 6), and others (n = 8). Immunohistochemical tissue staining was performed with a CCR4 monoclonal antibody on formalin-fixed paraffin-embedded tissue sections. CCR4 immunostaining was graded as percentage infiltrate, i.e. high (>25%) and low (≤25%), and the results were correlated with clinicopathological diagnoses. CCR4 expression was seen in 69% of the studied cases. Any CCR4 positivity was seen in all CD8+ MF cases, in 83% of CD30+ LPD cases, in 75% of AETCL cases, in 33% of GDTCL cases, and in none of the SPTCL cases. High CCR4 expression was seen in 79% of CD8+ MF cases versus 33% of CD30+ LPD cases, in 17% of GDTCL cases, and in 12.5% of AETCL cases. Patients with more advanced MF stage had higher CCR4 expression. CONCLUSIONS CCR4 immunohistochemistry may be an adjunct in distinguishing advanced CD8+ MF from other CD8+ CTCLs/LPDs. Although CCR4 expression may justify therapeutic targeting of this receptor in CD8+ MF, the role of such therapies in other CD8+ CTCLs/LPDs is not yet clear.
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Affiliation(s)
- Shamir Geller
- Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.,Department of Dermatology, Tel Aviv Sourasky Medical Centre, Tel Aviv, Israel
| | - Travis J Hollmann
- Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA
| | - Steven M Horwitz
- Department of Medicine, Lymphoma Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Patricia L Myskowski
- Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA
| | - Melissa Pulitzer
- Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA
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12
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13
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Mycosis fungoides in Taiwan shows a relatively high frequency of large cell transformation and CD56 expression. Pathology 2018; 50:718-724. [DOI: 10.1016/j.pathol.2018.08.008] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2018] [Revised: 08/17/2018] [Accepted: 08/22/2018] [Indexed: 12/23/2022]
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14
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Rovaris M, Colato C, Girolomoni G. Pediatric CD8+/CD56+ mycosis fungoides with cytotoxic marker expression: A variant with indolent course. J Cutan Pathol 2018; 45:782-785. [DOI: 10.1111/cup.13317] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2018] [Revised: 06/14/2018] [Accepted: 06/28/2018] [Indexed: 12/19/2022]
Affiliation(s)
- Marco Rovaris
- Department of Medicine, Section of Dermatology and Venereology; University of Verona; Verona Italy
| | - Chiara Colato
- Department of Pathology and Diagnostics, Section of Pathology; University of Verona; Verona Italy
| | - Giampiero Girolomoni
- Department of Medicine, Section of Dermatology and Venereology; University of Verona; Verona Italy
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15
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Nojima K, Namiki T, Miura K, Tanaka M, Yokozeki H. A case of CD8+
and CD56+
cytotoxic variant of poikilodermatous mycosis fungoides: Dermoscopic features of reticular pigmentation and vascular structures. Australas J Dermatol 2018. [DOI: 10.1111/ajd.12809] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Affiliation(s)
- Kohei Nojima
- Department of Dermatology; Graduate School of Medical and Dental Sciences; Tokyo Medical and Dental University; Tokyo Japan
| | - Takeshi Namiki
- Department of Dermatology; Graduate School of Medical and Dental Sciences; Tokyo Medical and Dental University; Tokyo Japan
| | - Keiko Miura
- Department of Pathology; Graduate School of Medical and Dental Sciences; Tokyo Medical and Dental University; Tokyo Japan
| | - Masaru Tanaka
- Department of Dermatology; Tokyo Women's Medical University Medical Center East; Tokyo Japan
| | - Hiroo Yokozeki
- Department of Dermatology; Graduate School of Medical and Dental Sciences; Tokyo Medical and Dental University; Tokyo Japan
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16
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Intraocular involvement of mycosis fungoides associated with immunophenotypic switch from CD4 + to CD8<sup/>. Blood 2018; 131:932-935. [PMID: 29298755 DOI: 10.1182/blood-2017-11-814194] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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17
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Martinez-Escala ME, Kantor RW, Cices A, Zhou XA, Kaplan JB, Pro B, Choi J, Guitart J. CD8 + mycosis fungoides: A low-grade lymphoproliferative disorder. J Am Acad Dermatol 2017; 77:489-496. [DOI: 10.1016/j.jaad.2017.05.015] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2017] [Revised: 05/01/2017] [Accepted: 05/15/2017] [Indexed: 11/17/2022]
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18
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Abstract
Primary cutaneous cytotoxic lymphomas are T-cell or natural killer-cell lymphomas that express 1 or more cytotoxic markers. These neoplasms constitute a spectrum of diseases. In this review, an overview of clinical, morphologic, and phenotypical features of each subtype is provided. Differential diagnosis is discussed with attention to scenarios in which diagnostic difficulties are most frequently encountered.
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Affiliation(s)
- Adriana García-Herrera
- Department of Pathology, Hospital Clínic de Barcelona, Villarroel, 170, Escalera 3, Planta 5, Barcelona 08036, Spain
| | - Eduardo Calonje
- Dermatopathology Laboratory, St John's Institute of Dermatology, St Thomas' Hospital, South Wing, Staircase C, Westminster Bridge Road, London SE1 7EH, UK.
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19
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Nikolaou V, Papadavid E, Patsatsi A, Siakantaris M, Economidi A, Marinos L, Koletsa T, Georgiou E, Pappa V, Stratigos A, Antoniou C. Prognostic indicators for mycosis fungoides in a Greek population. Br J Dermatol 2017; 176:1321-1330. [DOI: 10.1111/bjd.15000] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/20/2016] [Indexed: 12/12/2022]
Affiliation(s)
- V. Nikolaou
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - E. Papadavid
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - A. Patsatsi
- Aristotle University School of Medicine; Thessaloniki Greece
| | - M. Siakantaris
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - A. Economidi
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - L. Marinos
- Department of Hemopathology; Evangelismos Hospital; Athens Greece
| | - T. Koletsa
- Aristotle University School of Medicine; Thessaloniki Greece
| | - E. Georgiou
- Aristotle University School of Medicine; Thessaloniki Greece
| | - V. Pappa
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - A. Stratigos
- National and Kapodistrian University of Athens; Medical School; Athens Greece
| | - C. Antoniou
- National and Kapodistrian University of Athens; Medical School; Athens Greece
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20
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Thestrup-Pedersen K. Cutaneous T-Cell Lymphoma. A hypothesis on disease pathophysiology involving deficiency in DNA repair. J Eur Acad Dermatol Venereol 2016; 30:1682-1685. [DOI: 10.1111/jdv.13852] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2016] [Accepted: 05/24/2016] [Indexed: 11/28/2022]
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21
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Endo C, Naka Y, Miyagaki T, Fujita H, Sugaya M, Kawashima M, Tsunemi Y. Immunophenotypic shift from CD4+to CD8+in mycosis fungoides. Br J Dermatol 2016; 175:830-3. [DOI: 10.1111/bjd.14723] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Affiliation(s)
- C. Endo
- Department of Dermatology; Tokyo Women's Medical University; 8-1 Kawada-cho Shinjuku-ku Tokyo 162-8666 Japan
| | - Y. Naka
- Department of Dermatology; Tokyo Women's Medical University; 8-1 Kawada-cho Shinjuku-ku Tokyo 162-8666 Japan
| | - T. Miyagaki
- Department of Dermatology; Graduate School of Medicine and Faculty of Medicine; The University of Tokyo; Tokyo Japan
| | - H. Fujita
- Department of Dermatology; Nihon University School of Medicine; Tokyo Japan
| | - M. Sugaya
- Department of Dermatology; Graduate School of Medicine and Faculty of Medicine; The University of Tokyo; Tokyo Japan
| | - M. Kawashima
- Department of Dermatology; Tokyo Women's Medical University; 8-1 Kawada-cho Shinjuku-ku Tokyo 162-8666 Japan
| | - Y. Tsunemi
- Department of Dermatology; Tokyo Women's Medical University; 8-1 Kawada-cho Shinjuku-ku Tokyo 162-8666 Japan
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22
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Phenotypic Variation in Different Lesions of Mycosis Fungoides Biopsied Within a Short Period of Time From the Same Patient. Am J Dermatopathol 2016; 38:541-5. [DOI: 10.1097/dad.0000000000000493] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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23
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‘Could it be mycosis fungoides?’: an approach to diagnosing patch stage mycosis fungoides. J Hematop 2015. [DOI: 10.1007/s12308-015-0247-2] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
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24
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Hu SCS. Mycosis fungoides and Sézary syndrome: Role of chemokines and chemokine receptors. World J Dermatol 2015; 4:69-79. [DOI: 10.5314/wjd.v4.i2.69] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2015] [Revised: 03/16/2015] [Accepted: 04/09/2015] [Indexed: 02/06/2023] Open
Abstract
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma (CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skin-homing properties. Clinically and pathologically, mycosis fungoides can be categorized into patch, plaque and tumor stages. The clinical course of mycosis fungoides is usually chronic and indolent, but a proportion of patients may develop progressive disease with peripheral blood, lymph node and visceral organ involvement. Sézary syndrome is an aggressive leukemic form of CTCL characterized by a clonal population of malignant T cells in the peripheral blood. Various forms of skin-directed and systemic treatments are available for mycosis fungoides and Sézary syndrome. However, current treatments are generally not curative, and can only control the disease. Currently, the etiology and pathogenesis of mycosis fungoides and Sézary syndrome are not well defined. Proposed mechanisms include chronic antigenic stimulation by infectious agents, expression of specific adhesion molecules, altered cytokine production, mutations of oncogenes and tumor suppressor genes, and avoidance of apoptosis. In recent years, a number of chemokine receptors and their corresponding chemokine ligands have been found to contribute to the migration and survival of lymphoma cells in mycosis fungoides and Sézary syndrome, including CC chemokine receptor 4 (CCR4), CCR10, C-X-C chemokine receptor type 4 (CXCR4), CCR7, CCR3 and CXCR3. Since chemokines and chemokine receptors have been found to play important roles in the pathophysiology of mycosis fungoides and Sézary syndrome, they may be potentially useful targets for the development of new treatments for these diseases in the future.
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25
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Wobser M, Roth S, Reinartz T, Rosenwald A, Goebeler M, Geissinger E. CD68 expression is a discriminative feature of indolent cutaneous CD8-positive lymphoid proliferation and distinguishes this lymphoma subtype from other CD8-positive cutaneous lymphomas. Br J Dermatol 2015; 172:1573-1580. [PMID: 25524664 DOI: 10.1111/bjd.13628] [Citation(s) in RCA: 40] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/10/2014] [Indexed: 12/16/2022]
Abstract
BACKGROUND Indolent cutaneous CD8+ lymphoid proliferation is a recently described rare entity among cutaneous T-cell lymphomas that typically presents with solitary skin lesions at acral sites. Separation from otherwise aggressive T-cell lymphomas bearing a cytotoxic CD8+ phenotype is fundamental to avoid unnecessary harmful treatment. However, up to now, no reliable discriminative marker has been identified. OBJECTIVES Motivated by these diagnostic quandaries, we have analyzed a large series of archived formalin-fixed paraffin-embedded (FFPE) specimens of atypical CD8+ cutaneous infiltrates with clear-cut diagnosis and clinical follow-up (n = 44) including five cases of indolent CD8+ lymphoid proliferation by using immunohistochemistry with the aim of obtaining markers predictive of subtype assignment. RESULTS We identified exclusive expression of CD68 by lymphoma cells within the subgroup of indolent CD8+ lymphoid proliferation (5/5 cases). Specific CD68 expression in this entity was confirmed by the application of several monoclonal antibodies (KP1, PG-M1, KiM1P) against the CD68 molecule available for FFPE tissue. In contrast, none of the infiltrates of the other CD8+ cutaneous lymphoma entities stained positive for CD68 (0/39). CONCLUSIONS Based on these observations, we suggest CD68 as a new discriminative marker which is helpful in distinguishing indolent CD8+ lymphoid proliferation from other CD8+ cutaneous lymphomas in ambiguous cases.
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Affiliation(s)
- M Wobser
- Department of Dermatology, Comprehensive Cancer Center Mainfranken, University Hospital Wuerzburg, Josef-Schneider-Str. 2, Wuerzburg, 97080, Germany
| | - S Roth
- Institute of Pathology, Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany
| | - T Reinartz
- Department of Dermatology, Comprehensive Cancer Center Mainfranken, University Hospital Wuerzburg, Josef-Schneider-Str. 2, Wuerzburg, 97080, Germany
| | - A Rosenwald
- Institute of Pathology, Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany
| | - M Goebeler
- Department of Dermatology, Comprehensive Cancer Center Mainfranken, University Hospital Wuerzburg, Josef-Schneider-Str. 2, Wuerzburg, 97080, Germany
| | - E Geissinger
- Institute of Pathology, Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany
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26
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Jeon J, Kim JH, Ahn JW, Song HJ. Poikiloderma vasculare atrophicans showing features of ashy dermatosis in the beginning. Ann Dermatol 2015; 27:197-200. [PMID: 25834361 PMCID: PMC4377411 DOI: 10.5021/ad.2015.27.2.197] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2014] [Revised: 08/22/2014] [Accepted: 10/07/2014] [Indexed: 11/10/2022] Open
Abstract
Poikiloderma vasculare atrophicans (PVA) is a rare poikilodermatous variant of early-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectasia. In 2001, a 14-year-old male presented with asymptomatic brownish-gray polymorphic macules throughout the body with flexural accentuation. A skin biopsy showed increased melanophages with focal hydropic changes. Ashy dermatosis was considered a possible diagnosis. In 2005, the lesions began to show darkening and lichenification in the lower part of the trunk. In 2011, his skin showed definite poikilodermatous changes, and a biopsy showed band-like inflammatory infiltrations of atypical lymphocytes, epidermal atrophy, and epidermotropism of predominantly CD4-CD8+ atypical T cells. In addition, results of T-cell receptor gene rearrangement analysis were positive. Based on the aforementioned findings, he was diagnosed with PVA. If a patient shows long-standing and progressive hyperpigmentary skin changes, periodic follow-up and repeated skin biopsies are recommended to determine the underlying condition.
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Affiliation(s)
- Jiehyun Jeon
- Department of Dermatology, Korea University Guro Hospital, Seoul, Korea
| | - Joo Ha Kim
- Department of Dermatology, Korea University Guro Hospital, Seoul, Korea
| | - Jae Woo Ahn
- Department of Dermatology, Korea University Guro Hospital, Seoul, Korea
| | - Hae Jun Song
- Department of Dermatology, Korea University Guro Hospital, Seoul, Korea
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27
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Robson A, Assaf C, Bagot M, Burg G, Calonje E, Castillo C, Cerroni L, Chimenti N, Dechelotte P, Franck F, Geerts M, Gellrich S, Goodlad J, Kempf W, Knobler R, Massone C, Meijer C, Ortiz P, Petrella T, Pimpinelli N, Roewert J, Russell-Jones R, Santucci M, Steinhoff M, Sterry W, Wechsler J, Whittaker S, Willemze R, Berti E. Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop. Histopathology 2015; 67:425-41. [PMID: 24438036 DOI: 10.1111/his.12371] [Citation(s) in RCA: 65] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2013] [Accepted: 01/12/2014] [Indexed: 12/11/2022]
Abstract
AIMS Aggressive epidermotropic cutaneous CD8(+) lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8(+) lymphomas. METHODS AND RESULTS Sixty-one CD8(+) cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD8(+) lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and histologically have striking pagetoid epidermotrophism. A CD8(+) /CD45RA(+) /CD45RO(-) /CD2(-) /CD5(-) /CD56(-) phenotype, with one or more cytotoxic markers, was found in seven of 18 patients, with a very similar phenotype in the remainder. The tumours seldom involve lymph nodes, but mucosal and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD8(+) mycosis fungoides, lymphomatoid papulosis and Woringer-Kolopp disease presented the typical features well documented in the CD4(+) forms of those diseases. CONCLUSIONS Aggressive epidermotropic cutaneous CD8(+) lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.
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Affiliation(s)
| | - Chalid Assaf
- Department of Dermatology, Charité-University Medicine, Berlin, Germany
| | - Martine Bagot
- Department of Pathology, Universite Paris, Paris, France
| | - Gunter Burg
- Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
| | | | | | - Lorenzo Cerroni
- Department of Dermatology Medical, University of Graz, Graz, Austria
| | - Nicola Chimenti
- Department of Dermatology, University of L'Aquila, Rome, Italy
| | - Pierre Dechelotte
- Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
| | - Frederic Franck
- Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
| | - Maria Geerts
- Department of Dermatology, Ghent University Hospital, Gent, Belgium
| | - Sylke Gellrich
- Department of Dermatology, Charité-University Medicine, Berlin, Germany
| | - John Goodlad
- Department of Pathology, Western General Hospital, Edinburgh, UK
| | - Werner Kempf
- Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
| | - Robert Knobler
- Department of Dermatology, University of Vienna, Vienna, Austria
| | - Cesare Massone
- Department of Dermatology Medical, University of Graz, Graz, Austria
| | - Chris Meijer
- Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands
| | - Pablo Ortiz
- Hospital Universitario, Universidad Complutense, Madrid, Spain
| | - Tony Petrella
- Departmentof Pathology, Dijon's University Hospital, Dijon, France
| | - Nicola Pimpinelli
- Division of Dermatology, University of Florence Medical School, Florence, Italy
| | - Joclim Roewert
- Department of Dermatology, Charité-University Medicine, Berlin, Germany
| | | | - Marco Santucci
- Division of Pathological Anatomy, University of Florence, Florence, Italy
| | - Mattias Steinhoff
- Department of Dermatology, Charité-University Medicine, Berlin, Germany
| | - Wolfram Sterry
- Department of Dermatology, Charité-University Medicine, Berlin, Germany
| | - Janine Wechsler
- Department of Pathology Henri-Mondor Hospital, University Paris-Val-de-Marne, Paris, France
| | | | - Rein Willemze
- Department of Dermatology, Leiden University, Leiden, the Netherlands
| | - Emilio Berti
- Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy
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28
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Poppe H, Kerstan A, Böckers M, Goebeler M, Geissinger E, Rosenwald A, Hamm H. Childhood mycosis fungoides with a CD8+ CD56+ cytotoxic immunophenotype. J Cutan Pathol 2015; 42:258-64. [DOI: 10.1111/cup.12452] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2013] [Revised: 03/25/2014] [Accepted: 06/07/2014] [Indexed: 11/29/2022]
Affiliation(s)
- Heiko Poppe
- Department of Dermatology, Venereology and Allergology; University Hospital Würzburg; Würzburg Germany
| | - Andreas Kerstan
- Department of Dermatology, Venereology and Allergology; University Hospital Würzburg; Würzburg Germany
| | | | - Matthias Goebeler
- Department of Dermatology, Venereology and Allergology; University Hospital Würzburg; Würzburg Germany
| | - Eva Geissinger
- Institute of Pathology; University of Würzburg; Würzburg Germany
| | | | - Henning Hamm
- Department of Dermatology, Venereology and Allergology; University Hospital Würzburg; Würzburg Germany
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29
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Multicenter case series of indolent small/medium-sized CD8+ lymphoid proliferations with predilection for the ear and face. Am J Dermatopathol 2014; 36:402-8. [PMID: 24394306 DOI: 10.1097/dad.0b013e3182a74c7a] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
We report 7 cases of a CD8 lymphoid proliferation of the ear and face with a cytotoxic T-cell phenotype, but an indolent clinical course. All patients presented with stable or slowly growing asymptomatic lesions on the ear, nose, or lower eyelid. Histopathology showed a dense diffuse dermal infiltrate of small- to medium-sized atypical lymphocytes without destructive features. The lymphocytes were positive for CD3, CD8, β-F1, and TIA-1 and negative for CD4, CD30, CD56, granzyme B, and PD-1. Of note, the proliferation index was low in available cases. All patients remained in complete remission at median follow-up of 14 months regardless of treatment modality. Staging was negative for extracutaneous disease in all patients. The clinically indolent behavior and histopathologic phenotype together with a low proliferation index (10%-15%) emphasize the importance of accurate diagnosis and appropriate clinical management to avoid overtreatment and complications of therapy.
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30
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Karkouche R, Ingen-Housz-Oro S, Le Gouvello S, Charlotte F, Thomas M, Zehou O, Frenkel V, Boutboul D, Chosidow O, Caumes E, Gaulard P, Ortonne N. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with KIR3DL2 and NKp46 expression in a human immunodeficiency virus carrier. J Cutan Pathol 2014; 42:199-205. [PMID: 25407699 DOI: 10.1111/cup.12448] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2014] [Revised: 04/07/2014] [Accepted: 06/08/2014] [Indexed: 11/27/2022]
Abstract
Primary cutaneous aggressive epidermotropic T-cell lymphoma (PCAETCL) is a very rare lymphoma characterized by rapidly growing necrotic cutaneous lesions with an epidermotropic CD8+ T-cell neoplastic infiltrate observed histopathologically. It is associated with a very poor outcome, despite aggressive multi-agent chemotherapy. We report a 49-year-old human immunodeficiency virus (HIV)-infected patient who developed PCAETCL with associated marked vascular injury leading to diffuse purpuric and necrotic lesions complicated by recalcitrant hemophagocytic activation syndrome. The lymphoma strongly and diffusely expressed CD158k/KIR3DL2 at the protein and transcript level and NKp46 transcripts, in addition to CD8 and cytotoxic proteins. We observed a diffuse CD158k/KIR3DL2 protein expression in another case of PAETCL, not associated with immunodeficiency, which was used as a positive control. PCAETCL can develop in HIV-infected patients and may present in vasculitis-like fashion. The possible role of immunosuppression and/or HIV in oncogenesis can be postulated, as patients infected with HIV may develop anti-HIV cytotoxic CD8+ lymphoproliferations. The frequency of CD158k/KIR3DL2 and NKp46 expression in PCAECL remains to be studied in a series of cases, and may represent interesting targets for future treatments.
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Affiliation(s)
- Raymond Karkouche
- Département de Pathologie, Hôpital Henri Mondor, Université paris Est, Créteil, France
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31
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Kim M, Park MI, Lim M, Kim J. Cytotoxic Variant of Mycosis Fungoides with CD8+ CD56+ Phenotype: A Case Report and Review of Literature. KOREAN JOURNAL OF PATHOLOGY 2014; 48:390-3. [PMID: 25366078 PMCID: PMC4215968 DOI: 10.4132/koreanjpathol.2014.48.5.390] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/09/2013] [Accepted: 10/15/2013] [Indexed: 11/29/2022]
Affiliation(s)
- Meeran Kim
- Departments of Pathology, Chungnam National University Hospital, Daejeon, Korea
| | - Moon Il Park
- Departments of Pathology, Chungnam National University Hospital, Daejeon, Korea
| | - Myung Lim
- Departments of Dermatology, Chungnam National University Hospital, Daejeon, Korea
| | - Jinman Kim
- Departments of Pathology, Chungnam National University Hospital, Daejeon, Korea
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33
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Scarisbrick J, Kim Y, Whittaker S, Wood G, Vermeer M, Prince H, Quaglino P. Prognostic factors, prognostic indices and staging in mycosis fungoides and Sézary syndrome: where are we now? Br J Dermatol 2014; 170:1226-36. [DOI: 10.1111/bjd.12909] [Citation(s) in RCA: 100] [Impact Index Per Article: 9.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/14/2014] [Indexed: 12/28/2022]
Affiliation(s)
- J.J. Scarisbrick
- Department of Dermatology; University Hospital Birmingham; Birmingham U.K
| | - Y.H. Kim
- Stanford Cancer Centre & School of Medicine; Stanford CA U.S.A
| | - S.J. Whittaker
- Department of Dermatology; Guy's and St Thomas' NHS Trust; London U.K
| | - G.S. Wood
- Department of Dermatology; University of Wisconsin and Middleton VA Medical Center; Madison WI U.S.A
| | - M.H. Vermeer
- Department of Dermatology; Leiden University Medical Centre; Leiden the Netherlands
| | - H.M. Prince
- Peter MacCallum Cancer Centre and University of Melbourne; Melbourne VIC Australia
| | - P. Quaglino
- Department of Medical Sciences; Dermatologic Clinic; University of Torino; Turin Italy
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34
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35
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Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome). J Am Acad Dermatol 2014; 70:205.e1-16; quiz 221-2. [DOI: 10.1016/j.jaad.2013.07.049] [Citation(s) in RCA: 171] [Impact Index Per Article: 15.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2013] [Revised: 06/25/2013] [Accepted: 07/01/2013] [Indexed: 02/08/2023]
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36
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Wobser M, Petrella T, Kneitz H, Kerstan A, Goebeler M, Rosenwald A, Geissinger E. Extrafacial indolent CD8-positive cutaneous lymphoid proliferation with unusual symmetrical presentation involving both feet. J Cutan Pathol 2013; 40:955-61. [PMID: 24102688 DOI: 10.1111/cup.12213] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2013] [Revised: 07/09/2013] [Accepted: 08/17/2013] [Indexed: 12/28/2022]
Abstract
Indolent CD8+ cutaneous lymphoid proliferation represents a recently described entity among cutaneous T-cell lymphomas that typically presents with solitary skin lesions on the face or at acral sites and usually follows an indolent clinical course. Histopathologically, this entity is characterized by a dense dermal infiltrate of non-epidermotropic, small- to medium-sized pleomorphic CD8+ T-cells of the non-activated cytotoxic phenotype showing a clear-cut grenz zone and a low proliferation index. Distinction from otherwise aggressive T-cell lymphomas bearing a cytotoxic CD8+ phenotype is fundamental. We herein present an unusual case of indolent CD8+ cutaneous lymphoid proliferation presenting in bilateral symmetrical distribution on both feet and lacking the otherwise described grenz zone. Our case widens the spectrum of possible clinical and histomorphological variations of this entity. Taking into account the distinctive and unique clinical and microscopic features of all hitherto published cases of indolent CD8+ cutaneous lymphoid proliferation we suppose that this lymphoma subtype has to be included as a new and distinct entity in the World Health Organisation (WHO)-/European Organisation for Research and Treatment of Cancer (EORTC)-classification of cutaneous lymphomas.
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Affiliation(s)
- Marion Wobser
- Department of Dermatology, University Hospital Wuerzburg, Wuerzburg, Germany
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37
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Abstract
Primary cutaneous γδ T-cell lymphomas (PCGD-TCLs) are considered a subgroup of aggressive cytotoxic T-cell lymphomas (CTCLs). We have taken advantage of a new, commercially available antibody that recognizes the T-cell receptor-γ (TCR-γ) subunit of the TCR in paraffin-embedded tissue. We have analyzed a series of 146 primary cutaneous T-cell lymphomas received for consultation or a second opinion in the CNIO Pathology Department. Cases were classified according to the World Health Organization 2008 classification as mycosis fungoides (MF; n=96), PCGD-TCLs (n=5), pagetoid reticulosis (n=6), CD30(+) primary cutaneous anaplastic large cell lymphomas (n=5), primary cutaneous CD8 aggressive epidermotropic CTCLs (n=3), primary cutaneous CTCL, not otherwise specified (n=4), and extranodal nasal-type NK/T-cell lymphomas primarily affecting the skin or subcutaneous tissue (n=11). Sixteen cases of the newly named lymphomatoid papulosis type D (LyP-D; n=16) were also included. In those cases positive for TCR-γ, a further panel of 13 antibodies was used for analysis, including TIA-1, granzyme B, and perforin. Clinical and follow-up data were recorded in all cases. Twelve cases (8.2%) were positive for TCR-γ, including 5 PCGD-TCLs, 2 MFs, and 5 LyP-Ds. All 5 PCGD-TCL patients and 1 MF patient died of the disease, whereas the other MF patient and all those with LyP-D were alive. All cases expressed cytotoxic markers, were frequently CD3(+)/CD8(+), and tended to lose CD5 and CD7 expressions. Eight of 12 and 5 of 11 cases were CD30(+) and CD56(+), respectively. Interestingly, 5/12 TCR-γ-positive cases also expressed TCR-BF1. All cases analyzed were negative for Epstein-Barr virus-encoded RNA. In conclusion, TCR-γ expression seems to be rare and is confined to cytotoxic primary cutaneous TCLs. Nevertheless, its expression is not exclusive to PCGD-TCLs, as TCR-γ protein can be found in other CTCLs. Moreover, its expression does not seem to be associated with bad prognosis by itself, as it can be found in cases with good and bad outcomes.
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38
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Song SX, Willemze R, Swerdlow SH, Kinney MC, Said JW. Mycosis fungoides: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol 2013; 139:466-90. [PMID: 23525617 DOI: 10.1309/ajcpobdp2oqaj5br] [Citation(s) in RCA: 50] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
Abstract
Session 1 of the 2011 Workshop of the Society for Hematopathology and European Association for Haematopathology focused on mycosis fungoides (MF), the most common cutaneous lymphoma. The 62 cases in this case group demonstrated a wide spectrum of clinicopathologic features, including those seen in typical cases as well as those, by contrast, with atypical clinical history, morphology, immunophenotype, and/or genotype. Of the 62 cases, 27 (44%) were presented at the workshop and highlighted diagnostic challenges plus related issues. This report summarizes the approach recommended for making a confident diagnosis of MF and its clinically significant variants; emphasizes pitfalls in evaluating early MF, assessing nodal involvement, and diagnosing transformed MF; and discusses the relationship between MF and primary cutaneous CD30+ T-cell lymphoproliferative disorders. Last, Sézary syndrome is discussed, with concentration on those features distinct from MF.
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Affiliation(s)
- Sophie X. Song
- Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA
| | - Rein Willemze
- Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands
| | - Steven H. Swerdlow
- Department of Pathology, Division of Hematopathology, University of Pittsburgh School of Medicine, Pittsburgh, PA
| | - Marsha C. Kinney
- Department of Pathology, University of Texas Health Science Center, San Antonio, TX
| | - Jonathan W. Said
- Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA
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39
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Shiomi T, Monobe Y, Kuwabara C, Hayashi H, Yamamoto T, Sadahira Y. Poikilodermatous mycosis fungoides with a CD8+ CD56+ immunophenotype: a case report and literature review. J Cutan Pathol 2012; 40:317-20. [DOI: 10.1111/cup.12067] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2012] [Revised: 11/05/2012] [Accepted: 11/11/2012] [Indexed: 11/28/2022]
Affiliation(s)
- Tatsushi Shiomi
- Department of Pathology, Kawasaki Medical School; Kawasaki Hospital; Okayama Japan
- Department of Pathology; Kawasaki Medical School; Kurashiki Japan
| | - Yasumasa Monobe
- Department of Pathology, Kawasaki Medical School; Kawasaki Hospital; Okayama Japan
- Department of Pathology; Kawasaki Medical School; Kurashiki Japan
| | - Chiaki Kuwabara
- Department of Dermatology, Kawasaki Medical School; Kawasaki Hospital; Okayama Japan
| | - Haruko Hayashi
- Department of Dermatology, Kawasaki Medical School; Kawasaki Hospital; Okayama Japan
| | - Takenobu Yamamoto
- Department of Dermatology, Kawasaki Medical School; Kawasaki Hospital; Okayama Japan
| | - Yoshito Sadahira
- Department of Pathology; Kawasaki Medical School; Kurashiki Japan
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40
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Hyperpigmented mycosis fungoides: An unusual variant of cutaneous T-cell lymphoma with a frequent CD8+ phenotype. J Am Acad Dermatol 2012; 67:69-75. [DOI: 10.1016/j.jaad.2011.06.023] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2011] [Revised: 06/13/2011] [Accepted: 06/20/2011] [Indexed: 11/19/2022]
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Zeng W, Nava VE, Cohen P, Ozdemirli M. Indolent CD8-positive T-cell lymphoid proliferation of the ear: a report of two cases. J Cutan Pathol 2012; 39:696-700. [PMID: 22612273 DOI: 10.1111/j.1600-0560.2012.01917.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
The current classification of primary cutaneous T-cell lymphoma (CTCL) of the World Health Organization (WHO) includes primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma as a provisional entity awaiting cumulative data. Recent reports identify CD3/CD8-positive clonal T-cell lymphoid proliferations arising in the ear and nose that behave indolently and therefore defy currently established subclassification. Here, we report two cases of clonal CD8-positive/granzyme-B-negative T-cell lymphoid proliferations that arose in the ear and behaved indolently. Collectively, these cases suggest that an additional category of cutaneous indolent CD8-positive T-cell lymphoma may be necessary among the existing classification schemes.
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Affiliation(s)
- Weifen Zeng
- Department of Pathology, Georgetown University Hospital, Washington, DC 20007, USA
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42
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43
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Choi MS, Lee JB, Kim SJ, Lee SC, Won YH, Yun SJ. A case of poikiloderma vasculare atrophicans. Ann Dermatol 2011; 23 Suppl 1:S48-52. [PMID: 22028572 PMCID: PMC3199422 DOI: 10.5021/ad.2011.23.s1.s48] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2010] [Revised: 08/24/2010] [Accepted: 09/02/2010] [Indexed: 11/15/2022] Open
Abstract
Poikiloderma vasculare atrophicans (PVA) is a rare variant of mycosis fungoides, and is characterized by generalized hyperkeratotic scaly papules in net-like, retiform, or zebra-like patterns. A 59-year-old Korean woman presented with asymptomatic, erythematous-to-violaceous, reticulated confluent papules on the trunk and extremities. Skin lesions were initially limited to both thighs 25 years ago, and then spread slowly over her body. Histopathological examination showed band-like inflammatory infiltrations and epidermotropism consisting of mostly CD8+ lymphocytes. Based on the clinical manifestations and histological findings, the diagnosis of PVA was made. We herein report on a case of PVA, which featured a long-benign course without progression into the tumor stage over a period of 30 years.
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Affiliation(s)
- Myoung-Soon Choi
- Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea
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44
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Abbott RA, Sahni D, Robson A, Agar N, Whittaker S, Scarisbrick JJ. Poikilodermatous mycosis fungoides: A study of its clinicopathological, immunophenotypic, and prognostic features. J Am Acad Dermatol 2011; 65:313-319. [DOI: 10.1016/j.jaad.2010.05.041] [Citation(s) in RCA: 51] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2010] [Revised: 04/28/2010] [Accepted: 05/13/2010] [Indexed: 11/25/2022]
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45
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Meyerson HJ. A practical approach to the flow cytometric detection and diagnosis of T-cell lymphoproliferative disorders. ACTA ACUST UNITED AC 2010; 16:32-52. [PMID: 20858587 DOI: 10.1532/lh96.10001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
The flow cytometric analysis of T-cell malignancies is difficult due to the heterogeneity of T-cells and the lack of convenient methods to detect T-cell clonality. Neoplastic T-cells are most often detected by their altered level of surface antigen expression, and detection requires an extensive knowledge of the phenotype of normal T-lymphocytes. This review focuses on the methods to distinguish malignant T-cells from their normal counterparts and the phenotypic features of the T-cell lymphoproliferative disorders.
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Affiliation(s)
- Howard J Meyerson
- Department of Pathology and Ireland Cancer Center of Case Western Reserve University/University Hospitals Case Medical Center, Cleveland, Ohio 44106 , USA.
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46
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Alexandroff A, Flohr C, Johnston G. Updates from the British Association of Dermatologists 89th Annual Meeting, 7-10 July 2009, Glasgow, U.K. Br J Dermatol 2010; 163:27-37. [DOI: 10.1111/j.1365-2133.2010.09814.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
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