Published online Dec 6, 2021. doi: 10.12998/wjcc.v9.i34.10715
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
Processing time: 162 Days and 22 Hours
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.
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.