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ALK-rearranged histiocytosis

Monday 11 November 2019

ALK-positive histiocytosis

ALK-positive histiocytosis is a systemic histiocytic proliferation of early infancy with hepatosplenomegaly and dramatic hematological disturbances.

The clinicopathological spectrum of ALK-positive histiocytosis is broader than originally described, and this entity is characterized by frequent presence of KIF5B-ALK gene fusion.

It has been recommended that every unusual histiocytic proliferative disorder, especially disseminated lesions, be tested for ALK expression because of the potential efficacy of ALK inhibitor therapy in unresectable or disseminated disease. (30573850)