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10-10-2022 | Mastocytosis | Review

The international consensus classification of mastocytosis and related entities

Authors: Roos J. Leguit, Sa A. Wang, Tracy I. George, Alexandar Tzankov, Attilio Orazi

Published in: Virchows Archiv | Issue 1/2023

Abstract

Mastocytosis is a neoplasm characterized by a clonal proliferation of mast cells, which accumulate in one or multiple organs, associated with an extremely heterogeneous clinical presentation. The disease can be limited to the skin (cutaneous mastocytosis) that is mostly seen in childhood and usually behaves in a benign fashion. Adult patients most often present with systemic disease with or without skin lesions. This includes indolent forms such as indolent systemic mastocytosis and its subvariant bone marrow mastocytosis, and smoldering systemic mastocytosis as well as aggressive forms including aggressive systemic mastocytosis, systemic mastocytosis with an associated myeloid neoplasm (previously called systemic mastocytosis with an associated hematologic neoplasm), and mast cell leukemia. In addition, mast cell sarcoma is a rare aggressive form of mastocytosis that can present in the skin as well as at extracutaneous sites. This review article focuses on the updates in mastocytosis of the 2022 international consensus classification (ICC).

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Title: The international consensus classification of mastocytosis and related entities
Authors: Roos J. Leguit
Sa A. Wang
Tracy I. George
Alexandar Tzankov
Attilio Orazi
Publication date: 10-10-2022
Publisher: Springer Berlin Heidelberg
Keywords: Mastocytosis
Cutaneous Mastocytosis
Systemic Mastocytosis
Sarcoma
Leukemia
Published in: Virchows Archiv / Issue 1/2023
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-022-03423-3

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The international consensus classification of mastocytosis and related entities

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Keynote webinar | Spotlight on medication adherence

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Abstract

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