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Posted by on Jun 14, 2013 in Medical Journals |

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

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Cancer. 2011 Dec 15;117(24):5439-49. doi: 10.1002/cncr.26256. Epub 2011 Jun 20.

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

Quintás-Cardama A, Jain N, Verstovsek S.

Abstract

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.

Copyright © 2011 American Cancer Society.

PMID: 21692073 [PubMed – indexed for MEDLINE] Free full text