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Expert Opin Pharmacother. 2013 Oct;14(15):2033-45. doi: 10.1517/14656566.2013.824424.

Mastocytosis: update on pharmacotherapy and future directions.

Author information

1
Brigham and Women's Hospital, Division of Rheumatology, Allergy, Immunology, Mastocytosis Center, Department of Medicine , One Jimmy Fund Way, Smith Building, Room 626B, Boston, MA 02115 , USA.

Abstract

INTRODUCTION:

Mastocytosis is a disorder characterized by abnormal mast cell (MC) accumulation in skin and internal organs such as bone marrow. The disease follows a benign course in most patients with cutaneous and indolent systemic mastocytosis (SM); however, advanced variants associated with decreased life expectancy also exist. Pharmacotherapy of mastocytosis is aimed at the control of symptoms caused by MC mediator release, treatment of comorbidities and cytoreductive therapies in advanced variants.

AREAS COVERED:

This article will cover the general treatment principles of anti-MC mediator and cytoreductive therapies of mastocytosis. The literature discussed was retrieved with PubMed using the search terms 'treatment of mastocytosis,' 'mastocytosis antimediator therapy' and looking for important cross-references.

EXPERT OPINION:

Pharmacotherapy of mastocytosis should be individualized for each patient considering the category of disease, reduction of risk of anaphylaxis, constitutional symptoms and comorbidities including osteoporosis. Cytoreductive therapies are generally reserved for patients with aggressive mastocytosis (ASM), MC leukemia (MCL) and MC sarcoma (MCS); however, some patients with indolent disease and recurrent anaphylactic episodes not responsive to antimediator therapies may also be considered for cytoreduction on a case-by-case basis.

PMID:
24044484
PMCID:
PMC4362676
DOI:
10.1517/14656566.2013.824424
[Indexed for MEDLINE]
Free PMC Article

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