Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2021; 9(34): 10715-10722
Published online Dec 6, 2021. doi: 10.12998/wjcc.v9.i34.10715
Chronic lymphocytic leukemia/small lymphocytic lymphoma complicated with skin Langerhans cell sarcoma: A case report
Shao-Yan Li, Yan Wang, Li-Hua Wang
Shao-Yan Li, Yan Wang, Li-Hua Wang, Department of Dermatology, Jinan Central Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250013, Shandong Province, China
Author contributions: Li SY and Wang LH carried out the studies, participated in collecting data, and drafted the manuscript; Wang Y performed the statistical analysis and participated in its design; All authors read and approved the final manuscript.
Informed consent statement: Consent was obtained from relatives of the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Li-Hua Wang, MS, Department of Dermatology, Jinan Central Hospital, Cheeloo College of Medicine, Shandong University, No. 105 Jiefang Road, Lixia District, Jinan 250013, Province, China.
Received: June 20, 2021
Peer-review started: June 20, 2021
First decision: July 16, 2021
Revised: August 14, 2021
Accepted: September 16, 2021
Article in press: September 16, 2021
Published online: December 6, 2021

Langerhans cell sarcoma (LCS) is a rare malignancy with poor prognosis. LCS and chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) can occur in the same diseased tissues, such as lymph nodes or skin.


A 48-year-old female Han Chinese patient was admitted for generalized lymph node enlargement for 6 years and abdominal distension for 1 wk. She was diagnosed with small B-cell lymphoma (stage IV)/CLL (Benet stage B) and received chemotherapy. She started oral ibrutinib in February 2019. She was hospitalized on June 11, 2019, and a 1.5 cm × 1.5 cm dark-red nodule with ulceration scalp lesion was found. Biopsy revealed LCS but without CLL/SLL. She was diagnosed with CLL/SLL (Binet stage C, Rai stage IV) accompanied by secondary histiocytic sarcomas and skin LCS and received cyclophosphamide, doxorubicin, vincristine, dexamethasone, and etoposide but developed severe cytopenia. She ultimately refused treatments and discharged spontaneously. She died on September 12, 2019. The literature review showed that in patients with CLL/SLL, skin lesions of LCS are accompanied by CLL/SLL. This patient was different from the previously reported cases of skin LCS in patients with CLL/SLL.


In this patient, the skin lesion of LCS showed no concomitant CLL/SLL.

Keywords: Skin, Langerhans cell sarcomas, Chronic lymphocytic leukemia/small lymphocytic lymphoma, Ibrutinib, Case report

Core Tip: Langerhans cell sarcoma (LCS) is a rare Langerhans cell malignant tumor with a poor prognosis. Chronic lymphocytic leukemia (CLL) is a hematologic malignancy. We report a patient with a history of CLL/small lymphocytic lymphoma diagnosed with cutaneous LCS by skin biopsy that is different from previously reported cases. In the case report, LCS occurred on the skin, and there was no CLL/small lymphocytic lymphoma in the same lesion. A possible mechanism is that ibrutinib led to the activation of a proto-oncogene that resulted in the malignant transformation of dendritic cells. Still, this hypothesis will have to be explored.