Systémová mastocytóza [Systemic mastocytosis]

Details

Serval ID
serval:BIB_8965AC004A29
Type
Article: article from journal or magazin.
Collection
Publications
Title
Systémová mastocytóza [Systemic mastocytosis]
Journal
Vnitř Lék
Author(s)
Doubek M., Kozák T., Vašků V., Szturz Petr, Tichá M., Křen L.
Publication state
Published
Issued date
2010
Peer-reviewed
Oui
Volume
56
Number
Suppl 2
Pages
2S188-194
Language
Czech
Abstract
Systemic mastocytosis is a heterogenous disorder characterized by abnormal growth and accumulation of abnormal mast cells in one or more organs. Mast cells are derived from CD34+/ KIT+ pluripotent hematopoietic cells in the bone marrow. Most adult mastocytosis patients carry gain-of- function c- kit receptor mutations, most commonly D816V in the tyrosine kinase domain. The clinical phenotype of systemic mastocytosis is variable. Many of patients are suffering from urticaria pigmentosa, followed by the flushing, cramp-
ing, abdominal pain, diarrhea, bone pain and hepatosplenomegaly. Most adult patients have systemic disease (a condition generally confirmed by bone marrow biopsy). The natural history of systemic mastocytosis ranges from indolent forms to very aggressive subtypes.
Keywords
diagnostics, KIT, management, masitinib, systemic mastocytosis
Create date
08/01/2025 22:09
Last modification date
09/01/2025 7:05
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