Tags

Type your tag names separated by a space and hit enter

The new tool "KIT" in advanced systemic mastocytosis.

Abstract

Mastocytosis is a rare disease characterized by KIT-driven expansion and accumulation of neoplastic mast cells in various tissues. Although mediator symptoms related to mast cell activation can impose a symptom burden in cutaneous disease and across the spectrum of systemic mastocytosis subtypes, the presence of an associated hematologic neoplasm and/or organ damage denotes advanced disease and the potential for increased morbidity and mortality. In addition to the revised 2016 World Health Organization classification of mastocytosis, a new diagnostic and treatment toolkit, tethered to enhanced molecular characterization and monitoring, is poised to transform the management of patients with advanced systemic mastocytosis (advSM). Although the efficacy of midostaurin and novel selective KIT D816V inhibitors, such as avapritinib (BLU-285), have validated KIT as a therapeutic target, the clinical and biologic heterogeneity of advSM requires that we reimagine the blueprint for tackling these diseases and use tools that move beyond KIT-centric approaches.

Authors+Show Affiliations

Divisions of Hematology and. Medical Oncology, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA.Divisions of Hematology and.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

30504301

Citation

Shomali, William, and Jason Gotlib. "The New Tool "KIT" in Advanced Systemic Mastocytosis." Hematology. American Society of Hematology. Education Program, vol. 2018, no. 1, 2018, pp. 127-136.
Shomali W, Gotlib J. The new tool "KIT" in advanced systemic mastocytosis. Hematology Am Soc Hematol Educ Program. 2018;2018(1):127-136.
Shomali, W., & Gotlib, J. (2018). The new tool "KIT" in advanced systemic mastocytosis. Hematology. American Society of Hematology. Education Program, 2018(1), pp. 127-136. doi:10.1182/asheducation-2018.1.127.
Shomali W, Gotlib J. The New Tool "KIT" in Advanced Systemic Mastocytosis. Hematology Am Soc Hematol Educ Program. 2018 11 30;2018(1):127-136. PubMed PMID: 30504301.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The new tool "KIT" in advanced systemic mastocytosis. AU - Shomali,William, AU - Gotlib,Jason, PY - 2019/11/30/pmc-release PY - 2018/12/4/entrez PY - 2018/12/7/pubmed PY - 2019/1/11/medline SP - 127 EP - 136 JF - Hematology. American Society of Hematology. Education Program JO - Hematology Am Soc Hematol Educ Program VL - 2018 IS - 1 N2 - Mastocytosis is a rare disease characterized by KIT-driven expansion and accumulation of neoplastic mast cells in various tissues. Although mediator symptoms related to mast cell activation can impose a symptom burden in cutaneous disease and across the spectrum of systemic mastocytosis subtypes, the presence of an associated hematologic neoplasm and/or organ damage denotes advanced disease and the potential for increased morbidity and mortality. In addition to the revised 2016 World Health Organization classification of mastocytosis, a new diagnostic and treatment toolkit, tethered to enhanced molecular characterization and monitoring, is poised to transform the management of patients with advanced systemic mastocytosis (advSM). Although the efficacy of midostaurin and novel selective KIT D816V inhibitors, such as avapritinib (BLU-285), have validated KIT as a therapeutic target, the clinical and biologic heterogeneity of advSM requires that we reimagine the blueprint for tackling these diseases and use tools that move beyond KIT-centric approaches. SN - 1520-4383 UR - https://www.unboundmedicine.com/medline/citation/30504301/The_new_tool_"KIT"_in_advanced_systemic_mastocytosis_ L2 - http://www.asheducationbook.org/cgi/pmidlookup?view=long&pmid=30504301 DB - PRIME DP - Unbound Medicine ER -