Mastocytosis

Ayesha Abid; Michael A Malone; Katherine Curci

doi:10.1016/j.pop.2016.04.007

Mastocytosis

Prim Care. 2016 Sep;43(3):505-18. doi: 10.1016/j.pop.2016.04.007.

Authors

Ayesha Abid¹, Michael A Malone², Katherine Curci²

Affiliations

¹ Department of Family and Community Medicine, Penn State Milton S. Hershey Medical Center, 500 University Drive, Hershey, PA 17033, USA. Electronic address: aabid@hmc.psu.edu.
² Department of Family and Community Medicine, Penn State Milton S. Hershey Medical Center, 500 University Drive, Hershey, PA 17033, USA.

PMID: 27545739
DOI: 10.1016/j.pop.2016.04.007

Abstract

Mastocytosis is a rare disease caused by excessive production of mast cells. Clinical presentation is variable, often based on the type of mastocytosis, but in all types of mastocytosis there seems to be an increase in the risk of anaphylaxis. Systemic mastocytosis is diagnosed based on bone marrow biopsy. Treatment is variable based on the type of mastocytosis, but trigger avoidance and anaphylaxis treatment are mainstays. There are no therapies that change the natural course of mastocytosis. For cutaneous mastocytosis, treatment is conservative and aimed at symptom relief.

Keywords: Cutaneous mastocytosis; Diagnosis; Masocytosis; Pathophysiology; Review; Systemic mastocytosis; Treatment; Update.

Publication types

Review

MeSH terms

Anaphylaxis / etiology
Biopsy
Blood Cell Count
Blood Chemical Analysis
Humans
Mast Cells / metabolism
Mastocytosis, Cutaneous / complications
Mastocytosis, Cutaneous / diagnosis
Mastocytosis, Cutaneous / physiopathology*
Mastocytosis, Cutaneous / therapy
Mastocytosis, Systemic / complications
Mastocytosis, Systemic / diagnosis
Mastocytosis, Systemic / physiopathology*
Mastocytosis, Systemic / therapy
Mutation
Primary Health Care
Proto-Oncogene Proteins c-kit / genetics
Quality of Life
Tryptases / blood

Substances

Proto-Oncogene Proteins c-kit
Tryptases