Case Report: Rare Systemic and Aggressive ALK-Positive Histiocytosis With Recurrent Pancreatitis Treating by Alectinib

Yanchu Li; Changle Shi; Yu Wu; Mingmin He; Xueming Xia; Jie Liu; Yu Jiang

doi:10.3389/fmed.2022.840407

Case Report: Rare Systemic and Aggressive ALK-Positive Histiocytosis With Recurrent Pancreatitis Treating by Alectinib

Front Med (Lausanne). 2022 May 19:9:840407. doi: 10.3389/fmed.2022.840407. eCollection 2022.

Authors

Yanchu Li¹, Changle Shi², Yu Wu³, Mingmin He¹, Xueming Xia¹, Jie Liu¹, Yu Jiang¹

Affiliations

¹ Department of Head and Neck Oncology, West China Hospital of Sichuan University, Chengdu, China.
² Department of Pathology, West China Hospital of Sichuan University, Chengdu, China.
³ Department of Hematology, West China Hospital of Sichuan University, Chengdu, China.

Abstract

ALK-positive histiocytosis (APH) is a rare and recently described, solitary or generalized, histiocytic proliferative disorder with a characteristic gene translocation involving the fusion of the ALK gene at chromosome 2p23. To date, only 25 cases of APH have been reported. The patient presented with multiple nodules in the lung, liver, gallbladder, pancreas, kidney, and skin rashes, along with recurrent pancreatitis and cholecystitis. The histiocytes from the lesion were positive for CD68 and ALK and negative for S100 and CD1α. A reduced dose of the ALK inhibitor alectinib was administered rather than the standard dose of alectinib or chemotherapy because of recurrent pancreatitis, which has not been previously reported in APH cases. After 18 months of follow-up, the patient was maintained on alectinib, and a partial response (PR) was achieved.

Keywords: ALK inhibitor; ALK-positive histiocytosis; case report; distinct entity; pancreatitis.

Publication types

Case Reports