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

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 wit...

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Published inFrontiers in medicine Vol. 9; p. 840407
Main Authors Li, Yanchu, Shi, Changle, Wu, Yu, He, Mingmin, Xia, Xueming, Liu, Jie, Jiang, Yu
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 19.05.2022
Subjects
ALK inhibitor
ALK-positive histiocytosis
case report
distinct entity
Medicine
pancreatitis
case report
pancreatitis
ALK-positive histiocytosis
ALK inhibitor
distinct entity
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Summary: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 was administered rather than the standard dose of 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 , and a partial response (PR) was achieved.
Bibliography:This article was submitted to Hematology, a section of the journal Frontiers in Medicine
Edited by: Gerardo Ferrara, Ospedale Generale Provinciale Macerata, Italy
Reviewed by: Mauro Alaibac, University of Padua, Italy; Emilio Berti, University of Milan, Italy
These authors share first authorship
ISSN:2296-858X
2296-858X
DOI:10.3389/fmed.2022.840407