The international consensus classification of mastocytosis and related entities.

Autor: Leguit RJ; Department of Pathology, University Medical Center Utrecht, H04-312, POB 85500, 3508 GA, Utrecht, Netherlands., Wang SA; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., George TI; Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT, USA., Tzankov A; Institute of Pathology, University of Basel, University Hospital Basel, Schönbeinstrasse 40, 4031, Basel, Switzerland., Orazi A; Department of Pathology, PL Foster School of Medicine, Texas Tech University Health Sciences Center El Paso, 4800 Alberta Avenue, El Paso, TX, 79905, USA. Attilio.Orazi@ttuhsc.edu.
Jazyk: angličtina
Zdroj: Virchows Archiv : an international journal of pathology [Virchows Arch] 2023 Jan; Vol. 482 (1), pp. 99-112. Date of Electronic Publication: 2022 Oct 10.
DOI: 10.1007/s00428-022-03423-3
Abstrakt: 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).
(© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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