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Systemic mastocytosis: Management and prognosis

Authors
Cem Akin, MD, PhD
Jason Gotlib, MD, MS
Section Editor
Bruce S Bochner, MD
Deputy Editors
Anna M Feldweg, MD
Alan G Rosmarin, MD

INTRODUCTION

Systemic mastocytosis (SM) consists of a group of disorders exhibiting excessive mast cell accumulation, typically in bone marrow and other extracutaneous tissues (table 1). The general management and pharmacologic treatment of SM will be discussed in this topic review. The clinical manifestations, classification, pathogenesis, evaluation, and diagnosis of SM are reviewed separately:

(See "Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations".)

(See "Mastocytosis (cutaneous and systemic): Evaluation and diagnosis in adults".)

(See "Mastocytosis (cutaneous and systemic): Evaluation and diagnosis in children".)

OVERVIEW

Systemic mastocytosis (SM) is a heterogenous disorder. Patients are classified into one of five clinical subtypes (also called variants), which differ in clinical presentation, treatment, and prognosis (table 1):

                                              

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Literature review current through: Dec 2016. | This topic last updated: Tue Dec 20 00:00:00 GMT+00:00 2016.
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