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Other Neoplasms of Myeloid Origin: Histiocytic/Dendritic-Cell and Mast Cell Neoplasms
Abstract
Other rare neoplasms may arise from myeloid progenitors, including Histiocytic/ Dendritic Neoplasms and mast cell neoplasms. The Classification of both categories in the 5th edition of WHO has some modifications with newly introduced entities. Histiocytic neoplasms exhibit diverse somatic oncogenic alterations. Their clinical presentation ranges from incidental findings to critical illness with severe organ dysfunction. Each subtype has distinct morphologic and immunophenotypic features. However, some overlap exists between the different subtypes. Mastocytosis involves abnormal mast cells accumulating in various organs. It develops as a clonal expansion of mast cells derived by constitutive activation of the KIT receptor. The clinical course varies from asymptomatic to diffuse systemic involvement with comorbidities mainly related to mediators' release.
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