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Advances and controversies in the diagnosis, pathogenesis, and treatment of systemic mastocytosis
Author(s) -
QuintásCardama Alfonso,
Jain Nitin,
Verstovsek Srdan
Publication year - 2011
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/cncr.26256
Subject(s) - systemic mastocytosis , pathogenesis , medicine , haematopoiesis , bone marrow , somatic cell , viral oncogene , immunology , cancer , cancer research , pathology , gene , biology , genetics , stem cell
The term systemic mastocytosis (SM) encompasses a group of hematopoietic malignancies characterized by excessive proliferation of neoplastic mast cells that accumulate in the bone marrow and visceral organs. Most patients with SM, particularly those who present with aggressive clinical courses, carry somatic mutations of the v‐kit Hardy‐Zuckerman 4 feline sarcoma viral oncogene homolog ( KIT ) gene. KIT mutations are considered central events in the pathogenesis of SM and serve as diagnostic markers and putative therapeutic targets. The heterogeneity in the clinical course of patients with SM and recent advances in the genetic and immunophenotypic characterization of neoplastic mast cells may help to improve current diagnostic, taxonomic, and therapeutic approaches in SM. Cancer 2011;. © 2011 American Cancer Society.