Advances and controversies in the diagnosis, pathogenesis, and treatment of systemic mastocytosis

被引:18
|
作者
Quintas-Cardama, Alfonso [1 ]
Jain, Nitin [1 ]
Verstovsek, Srdan [1 ]
机构
[1] Univ Texas MD Anderson Canc Ctr, Dept Leukemia, Houston, TX 77030 USA
关键词
diagnosis; KIT; midostaurin; prognosis; systemic mastocytosis; TYROSINE KINASE INHIBITOR; NEOPLASTIC MAST-CELLS; PROTOONCOGENE C-KIT; PHASE-II; IN-VITRO; CUTANEOUS MASTOCYTOSIS; DASATINIB BMS-354825; ACTIVATING MUTATION; ANTITUMOR-ACTIVITY; IMATINIB MESYLATE;
D O I
10.1002/cncr.26256
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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;117:5439-49. (C) 2011 American Cancer Society.
引用
收藏
页码:5439 / 5449
页数:11
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