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 in | Frontiers in medicine Vol. 9; p. 840407 |
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Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
Published |
Switzerland
Frontiers Media S.A
19.05.2022
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Subjects | |
Online Access | Get full text |
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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. |
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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 |