Mastocytosis: molecular mechanisms and clinical disease heterogeneity

Citation
Dd. Metcalfe et C. Akin, Mastocytosis: molecular mechanisms and clinical disease heterogeneity, LEUK RES, 25(7), 2001, pp. 577-582
Citations number
33
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Onconogenesis & Cancer Research
Journal title
LEUKEMIA RESEARCH
ISSN journal
0145-2126 → ACNP
Volume
25
Issue
7
Year of publication
2001
Pages
577 - 582
Database
ISI
SICI code
0145-2126(200107)25:7<577:MMMACD>2.0.ZU;2-F
Abstract
Systemic mastocytosis has one unifying feature: an unexplained and patholog ic increase in mast cells in specific tissues. This observation, along with clinical disease heterogeneity has long suggested that mastocytosis is a d isease of complex etiology. At the same time, the last decade has witnessed significant progress in identifying the critical elements that regulate ma st cell growth and development. Human mast cells are now known to arise fro m CD34(+) progenitors, particularly under the influence of stem cell factor (SCF). This information in turn led to the critical observation that a sub stantial number of patients with mastocytosis exhibit activating mutations in c-kit, the receptor for SCF. And while this observation may well be key in understanding mastocytosis, this mutation alone does not explain all het erogeneity. It now appears that other influences such as genetic polymorphi sms within the host may influence the course of disease in those with KIT m utations; and that the search for additional molecular events capable of cr eating disease diversity must continue. Published by Elsevier Science Ltd.