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Domínguez Cañete JJ, Platas Moreno I. [Duodenitis Russell bodies. Review of the entity and associations beyond H. pylori]. REVISTA ESPANOLA DE PATOLOGIA : PUBLICACION OFICIAL DE LA SOCIEDAD ESPANOLA DE ANATOMIA PATOLOGICA Y DE LA SOCIEDAD ESPANOLA DE CITOLOGIA 2024; 57:190-197. [PMID: 38971619 DOI: 10.1016/j.patol.2024.02.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/30/2023] [Revised: 01/24/2024] [Accepted: 02/13/2024] [Indexed: 07/08/2024]
Abstract
Plasma cells known as "Mott cells" present non-secretable accumulations of immunoglobulins called "Russell bodies". Its presence is related to hematological neoplasms, but it can appear in chronic inflammatory processes. The most common occurrence within the digestive tract is the gastric antrum associated with H. pylori infection. Our patient is added the rare extragastric cases where the association with H. pylori is inconsistent. We have found a frequent appearance of lower digestive and urological neoplasms in relation to these cases, justified by the expression of circulating cytokines in the tumor area that lead to the overactivation of plasma cells. This possible association could lead us to know data about the tumor environment and serve us for early diagnosis or future therapeutic targets.
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Luo S, Huang X, Li Y, Wang J. Surveillance of Russell body inflammation of the digestive tract: a case report and review of literature. Diagn Pathol 2022; 17:67. [PMID: 36002890 PMCID: PMC9404552 DOI: 10.1186/s13000-022-01242-3] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2021] [Accepted: 06/15/2022] [Indexed: 12/04/2022] Open
Abstract
Introduction Russell body inflammation of the digestive tract (RBIDT) is a rare chronic inflammation of the digestive tract mucosa that commonly presents as Russell body gastritis (RBG). This disease is usually associated with Helicobacter pylori (HP) infection. However, it can also occur in individuals without HP infection and with specific immune profiles, as seen in HIV and hepatitis C infections. The aetiology and pathogenesis of this disease remain controversial. Given the rarity of the disease and the diversity of the immunophenotypes, there is a high probability of misdiagnosis. Case presentation A male patient with RBG and HP infection was included in this study. The case of RBG with an unusual morphology of Mott cells that mimics stamped ring cells.Endoscopy performed during the follow-up revealed regression of the lesion 1 week after anti-HP treatment. Conclusions A case of RBG with a high likelihood of misdiagnosis of signet ring cell carcinoma (SRC) has been reported in this study along with a review of the relevant literature and an overview of RBIDT.
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Affiliation(s)
- Shuai Luo
- Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, P.R. China
| | - Xiang Huang
- Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, P.R. China
| | - Yao Li
- Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, P.R. China
| | - Jinjing Wang
- Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, P.R. China.
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Peruhova M, Peshevska-Sekulovska M, Georgieva V, Panayotova G, Dikov D. Surveilling Russell body Helicobacter pylori-negative gastritis: A case report and review of literature. World J Gastroenterol 2020; 26:5050-5059. [PMID: 32952348 PMCID: PMC7476171 DOI: 10.3748/wjg.v26.i33.5050] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/20/2020] [Revised: 08/03/2020] [Accepted: 08/25/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Russell body gastritis (RBG) is very rare type of chronic inflammation of gastric mucosa. The pathologic hallmark of the disease is Russell bodies (RB) which represent accumulation of eosinophilic cytoplasmic inclusions in endoplasmic reticulum of mature plasma cells (Mott cells). Most published cases are associated with Helicobacter pylori (H. pylori) infection because of correlation between plasma cell activation and antigenic stimulation. There are insufficient data about H. pylori-negative RBG and very little is known about the natural course of the disease.
CASE SUMMARY A 51-year-old male patient underwent endoscopic screening for mild iron deficiency anemia. Gastroscopy revealed diffuse hyperemia, edema and nodularity of the fundic and corpus mucosa. Due to non-specific endoscopic findings and iron-deficiency anemia our preliminary diagnosis was diffuse type of gastric carcinoma or gastric lymphoma. Biopsy specimens of gastric mucosa showed inflammatory infiltrate rich in Mott cells, consisting entirely of cytoplasmic RB. Absence of nuclear atypia and mitosis of the plasma cells, polyclonal pattern of the Mott cells and negative staining for cytokeratins favored diagnosis of RBG. The patient was treated with proton-pump inhibitor for 8 wk. Long-term clinical and endoscopic surveillance was scheduled. Albeit, there was no improvement in endoscopic features of the gastric mucosa in three consecutive gastroscopies, histopathological findings demonstrated that the chronic inflammatory infiltrate in the fundic mucosa is less pronounced, rich in plasma cells, with almost absent RB and Mott cells.
CONCLUSION The prognosis of this entity is uncertain, that is why these patients are subjects of continuous follow up.
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Affiliation(s)
- Milena Peruhova
- Department of Gastroenterology, University Hospital Lozenetz, Sofia 1407, Bulgaria
| | | | - Viktoriya Georgieva
- Department of Gastroenterology, University Hospital Lozenetz, Sofia 1407, Bulgaria
| | - Gabriela Panayotova
- Department of Gastroenterology, University Hospital Lozenetz, Sofia 1407, Bulgaria
| | - Dorian Dikov
- Department of General and Clinical pathology, Grand Hôpital de l'Est Francilien, Jossigny 77600, France
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Analysis of clinical and histopathological findings in Russell body gastritis and duodenitis. Ann Diagn Pathol 2019; 40:66-71. [PMID: 31031217 DOI: 10.1016/j.anndiagpath.2019.04.003] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2018] [Revised: 03/09/2019] [Accepted: 04/02/2019] [Indexed: 02/06/2023]
Abstract
INTRODUCTION Russell body gastritis is considered as a rare, benign, incidental finding characterized by dense accumulation of plasma cells containing Russell bodies in the lamina propria. In this study, clinical and histopathological features of 12 cases of Russell body gastritis/duodenitis were presented. MATERIALS AND METHODS Clinical data, histopathological findings including Helicobacter pylori infection, Sydney system classification, Russell body density and immunohistochemical findings were evaluated in 11 gastric and 1 duodenal mucosal biopsy from 11 patients. RESULTS Six cases were male, 5 were female and the mean age was 72 (44-87). The most common site was antrum (10/12), one case was located in cardia and one in heterotopic gastric mucosa of duodenal bulb. H. pylori was detected in half of the cases. One of the cases was accompanied by gastric tubular adenoma, one by gastric well-differentiated adenocarcinoma and one by plasma cell neoplasm. In all cases, globules were positive with PAS stain. CONCLUSION Russell body gastritis must be kept in mind while reporting endoscopic biopsies because this entity may be misdiagnosed as signet ring carcinoma and may be associated with neoplasms. Absence of nuclear atypia, mucin stains, cytokeratins, plasma cell and hematolymphoid antigen markers are useful in differential diagnosis. Associated H. pylori infection, as well as rarely carcinomas, adenomas and plasma cell neoplasms, may be observed.
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Cortes-Santiago N, Schady DA. Kappa restricted Russell body gastroenteritis in two pediatric patients. HUMAN PATHOLOGY: CASE REPORTS 2018. [DOI: 10.1016/j.ehpc.2017.10.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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Yorita K, Iwasaki T, Uchita K, Kuroda N, Kojima K, Iwamura S, Tsutsumi Y, Ohno A, Kataoka H. Russell body gastritis with Dutcher bodies evaluated using magnification endoscopy. World J Gastrointest Endosc 2017; 9:417-424. [PMID: 28874963 PMCID: PMC5565508 DOI: 10.4253/wjge.v9.i8.417] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/29/2016] [Revised: 03/13/2017] [Accepted: 04/24/2017] [Indexed: 02/06/2023] Open
Abstract
Russell body gastritis (RBG) is an unusual type of chronic gastritis characterized by marked infiltration of Mott cells, which are plasma cells filled with spherical eosinophilic bodies referred to as Russell bodies. It was initially thought that Helicobacter pylori (H. pylori) infection was a major cause of RBG and that the infiltrating Mott cells were polyphenotypic; however, a number of cases of RBG without H. pylori infection or with monoclonal Mott cells have been reported. Thus, diagnostic difficulty exists in distinguishing RBG with monoclonal Mott cells from malignant lymphoma. Here, we report an unusual case of an 86-year-old-Japanese man with H. pylori-positive RBG. During the examination of melena, endoscopic evaluation confirmed a 13-mm whitish, flat lesion in the gastric antrum. Magnification endoscopy with narrow-band imaging suggested that the lesion was most likely a poorly differentiated adenocarcinoma. Biopsy findings were consistent with chronic gastritis with many Mott cells with intranuclear inclusions referred to as Dutcher bodies. Endoscopic submucosal dissection confirmed the diagnosis of RBG with kappa-restricted monoclonal Mott cells. Malignant lymphoma was unlikely given the paucity of cytological atypia and Ki-67 immunoreactivity of monoclonal Mott cells. This is the first reported case of RBG with endoscopic diagnosis of malignant tumor and the presence of Dutcher bodies.
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Joo M. Rare Gastric Lesions Associated with Helicobacter pylori Infection: A Histopathological Review. J Pathol Transl Med 2017; 51:341-351. [PMID: 28592787 PMCID: PMC5525039 DOI: 10.4132/jptm.2017.04.03] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2016] [Accepted: 04/03/2017] [Indexed: 12/20/2022] Open
Abstract
Helicobacter pylori infection is associated with chronic gastritis, peptic ulcer disease, gastric adenocarcinoma, and mucosa-associated lymphoid tissue lymphoma. However, some rare gastric lesions exhibiting distinctive histological features may also be associated with H. pylori infection, including lymphocytic gastritis, granulomatous gastritis, Russell body gastritis, or crystal-storing histiocytosis. Although diverse factors can contribute to their development, there is convincing evidence that H. pylori infection may play a pathogenic role. These findings are mainly based on studies in patients with these lesions who exhibited clinical and histological improvements after H. pylori eradication therapy. Thus, H. pylori eradication therapy might be indicated in patients with no other underlying disease, particularly in countries with a high prevalence of H. pylori infection. This review describes the characteristic histological features of these rare lesions and evaluates the evidence regarding a causative role for H. pylori infection in their pathogenesis.
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Affiliation(s)
- Mee Joo
- Department of Pathology, Inje University Ilsan Paik Hospital, Goyang, Korea
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Muthukumarana V, Segura S, O'Brien M, Siddiqui R, El-Fanek H. "Russell Body Gastroenterocolitis" in a Posttransplant Patient: A Case Report and Review of Literature. Int J Surg Pathol 2015; 23:667-72. [PMID: 26310272 DOI: 10.1177/1066896915601893] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Russell bodies represent a cellular response to overstimulation of plasma cells, leading to the accumulation of abundant, nondegradable, condensed immunoglobulin in dilated rough endoplasmic reticulum cisternae. Russell body gastritis was first described 1998 by Tazawa and Tsutsumi. Since then only 39 cases involving the gastrointestinal tract have been reported in English literature, which include Russell body gastritis, duodenitis, and esophagitis. We report a case of a 44-year-old female with a history of diabetes mellitus, status post kidney and pancreas transplant who presented with multiple episodes of watery diarrhea associated with abdominal pain, nausea, and vomiting. Upper gastroendoscopic examination showed diffuse mild erythema in the gastric body and a clean-based duodenal ulcer. Lower gastroendoscopic examination was normal. Examination of multiple biopsies from duodenal, gastric, terminal ileum, and colonic mucosae revealed numerous plasma cells with abundant eosinophilic granular cytoplasm (Russell bodies) and eccentric nuclei, highlighted by PAS stain and CD 138 plasma cell marker. Helicobacter pylori stains were performed on gastric biopsies and were negative for organisms. To date, there are no cases described in English literature with multifocal Russell body infiltrates in gastrointestinal tract in a single patient including ileum and/or colon. This makes our case the first to be reported with these unique findings; thus, the spectrum of Russell body-associated chronic inflammation of the gastrointestinal tract would be more suitably referred to as "Russell body gastroenterocolitis."
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Affiliation(s)
| | - Sheila Segura
- Western Connecticut Health Network, Danbury, CT, USA
| | | | - Rina Siddiqui
- Western Connecticut Health Network, Danbury, CT, USA
| | - Hani El-Fanek
- Western Connecticut Health Network, Danbury, CT, USA
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9
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Russell body gastritis/duodenitis: a case series and description of immunoglobulin light chain restriction. Clin Res Hepatol Gastroenterol 2014; 38:e89-97. [PMID: 25001185 DOI: 10.1016/j.clinre.2014.05.008] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2014] [Revised: 05/21/2014] [Accepted: 05/28/2014] [Indexed: 02/04/2023]
Abstract
Russell body esophago-gastro-duodenitis is an unusual form of chronic inflammation, with only 22 cases being reported in PubMed. However, the prevalence and clinical significance remain unknown. This report describes the clinico-pathological characteristics of nine cases of Russell body gastritis (RBG) and one case of Russell body duodentitis (RBD), with nonspecific endoscopic appearance. The Mott cells (plasma cells with Russell bodies) showed κ light chain restriction in eight gastritis cases and λ light chain restriction in the duodentitis case, and there were no histological features that suggested lymphoma. Thus, a diagnosis of monoclonal RBG/RBD was made. Helicobacter pylori infection was found in 55.6% of RBG cases and in the RBD case. And, the clinical follow-up evaluations were uneventful. This report is the first study to describe this benign disease entity with monoclonality on a large-scale basis. In addition, the monoclonality of Mott cells cannot be used as evidence of an existing neoplastic lesion, and taken together, these findings may indicate a reactive process.
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Beghini M, Montes JMC, Rodrigues DBR, Teixeira VPA, Pereira SADL. Reduction of immunity in HIV-infected individuals: can fibrosis induce hypoplasia in palatine and lingual tonsils of individuals with HIV infection? Pathol Res Pract 2014; 211:27-35. [PMID: 25441659 DOI: 10.1016/j.prp.2014.06.032] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2013] [Revised: 06/18/2014] [Accepted: 06/24/2014] [Indexed: 11/16/2022]
Abstract
The role of tonsils in oral immunity has been described. However, the pathogenesis of HIV infection in these organs is still unclear. The aim of this study is to perform histological and immunohistochemical analysis of the palatine and lingual tonsils of autopsied individuals with or without HIV infection. Twenty-six autopsied individuals with HIV infection (HI) (n=13) and without HIV infection (CO) (n=13) were selected. Palatine and lingual tonsil fragments were collected for histological and immunohistochemical analysis. We found in the HI group a higher frequency of hyaline degeneration in both palatine and lingual tonsils; smaller follicle areas, and a higher percentage of collagen in comparison with the CO group. In the HI group, there was higher density of blood vessels in palatine tonsils than in the CO group. In the HI group, there were significant positive correlations between palatine and lingual tonsils and the area of lymphoid follicles, and between the percentage of blood vessels and collagen in palatine tonsils. In addition, there was a significant negative correlation between the percentage of collagen and lymphoid follicle area in both palatine and lingual tonsils in the HI group. These findings suggest that the immune functions of these tonsils are prejudiced by fibrosis. Therapies to reduce the neoformation of collagen are required to improve immune function of organs against pathogens.
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Affiliation(s)
- Marcela Beghini
- General Pathology Division, Federal University of Triangulo Mineiro (UFTM), Uberaba, MG, Brazil
| | - Jean M C Montes
- Laboratory of Biopathology and Molecular Biology, University of Uberaba (UNIUBE), Uberaba, MG, Brazil
| | - Denise B R Rodrigues
- Laboratory of Biopathology and Molecular Biology, University of Uberaba (UNIUBE), Uberaba, MG, Brazil; Cefores, Federal University of Triangulo Mineiro (UFTM), Uberaba, MG, Brazil
| | - Vicente P A Teixeira
- General Pathology Division, Federal University of Triangulo Mineiro (UFTM), Uberaba, MG, Brazil
| | - Sanívia A de Lima Pereira
- Laboratory of Biopathology and Molecular Biology, University of Uberaba (UNIUBE), Uberaba, MG, Brazil; Cefores, Federal University of Triangulo Mineiro (UFTM), Uberaba, MG, Brazil.
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Klair JS, Girotra M, Kaur A, Aduli F. Helicobacter pylori-negative Russell body gastritis: does the diagnosis call for screening for plasmacytic malignancies, especially multiple myeloma? BMJ Case Rep 2014; 2014:bcr-2013-202672. [PMID: 24671320 DOI: 10.1136/bcr-2013-202672] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Russell body gastritis (RBG) is a rare entity with unestablished pathophysiology, endoscopic findings, clinical manifestations and treatments. Literature is scarce on this clinical entity with unclear clinical significance. Of 18 cases reported, 12 tested (+) for Helicobacter pylori and improved with treatment, but it remains unclear whether this link is coincidental or bears some clinical significance. We describe a case of elderly woman who had a follow-up oesophagogastroduodenoscopy for chronic peptic ulcers, and biopsy showed positive immunohistochemical stains for κ and λ, indicating a polytypic population of plasma cells. Immunostaining for H pylori was negative. Biopsies were also (-) for gastric carcinoma, lymphoma and plasmacytoma. Considering her RGB-suggestive histology and her symptoms of bone pains and anaemia, multiple myeloma screening was considered clinically relevant. The purpose of this review was to educate clinicians and gastroenterologists about this unique entity and explore its association with multiple myeloma or other plamacytic malignancies.
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Affiliation(s)
- Jagpal Singh Klair
- Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
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12
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Russell body gastroenteritis: an aberrant manifestation of chronic inflammation in gastrointestinal mucosa. Case Rep Med 2013; 2013:797264. [PMID: 24198839 PMCID: PMC3808711 DOI: 10.1155/2013/797264] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2013] [Accepted: 09/11/2013] [Indexed: 12/18/2022] Open
Abstract
First described in 1998, Russell body gastritis is a rare chronic inflammatory condition characterized by abundant intramucosal polyclonal plasma cells, which contain intracytoplasmic eosinophilic globules of immunoglobulins (Russell bodies) that displace the nucleus, with an accompanying chronic inflammatory infiltrate. Russell bodies represent a cellular response to overstimulation of plasma cells, leading to the accumulation of abundant, nondegradable, condensed immunoglobulin in dilated rough endoplasmic reticulum cisternae. Russell body gastritis usually occurs in the gastric antrum, but two cases of Russell body duodenitis have been recently described. Herein, we report an unusual case of Barrett esophagus with prominent lymphoplasmacytic infiltration and Russell bodies, which expands the current spectrum of Russell body gastritis/duodenitis. Given the various anatomic locations in which Russell body gastritis may arise, we suggest that “Russell body gastroenteritis” may be a more appropriate designation for this uncommon reactive condition.
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Takahashi Y, Shimizu S, Uraushihara K, Fukusato T. Russell body duodenitis in a patient with retroperitoneal metastasis of ureteral cancer. World J Gastroenterol 2013; 19:125-8. [PMID: 23326174 PMCID: PMC3542750 DOI: 10.3748/wjg.v19.i1.125] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2012] [Revised: 09/26/2012] [Accepted: 10/16/2012] [Indexed: 02/06/2023] Open
Abstract
Russell bodies are globular and eosinophilic inclusion bodies in the cytoplasm of mature plasma cells. Plasma cells whose cytoplasm is filled with Russell bodies are designated as Mott cells. Russell body duodenitis (RBD) is a unique form of chronic duodenitis that is characterized by infiltration of numerous Mott cells. RBD is very rare; only two cases have been reported to date. In this paper, we report a case of RBD in a patient with retroperitoneal metastasis of ureteral cancer. A 77-year-old man was admitted to our hospital complaining of appetite loss, vomiting, and upper abdominal distension. He had undergone left nephroureterectomy for ureteral cancer 4 years earlier. Upper digestive tract endoscopy revealed edema, stenosis, and punctate redness of the mucosa of the duodenum, and a biopsy was performed. Histological analysis showed that numerous Mott cells had infiltrated the lamina propria mucosae, and the condition was diagnosed as RBD. A mass lesion in the retroperitoneum adjacent to the duodenum was detected by abdominal computed tomography, and was diagnosed as metastatic urothelial carcinoma by biopsy. It is possible that chemokines produced by tumor cells caused RBD in this case.
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Choi J, Eun Lee H, Byeon SJ, Nam KH, Kim MA, Kim WH. Russell body gastritis presented as a colliding lesion with a gastric adenocarcinoma: A case report. ACTA ACUST UNITED AC 2012. [DOI: 10.1111/j.1755-9294.2012.01127.x] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
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