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Systemic mastocytosis: Determining the subtype of disease

Cem Akin, MD, PhD
Jason Gotlib, MD, MS
Section Editor
Sarbjit Saini, MD
Deputy Editors
Anna M Feldweg, MD
Alan G Rosmarin, MD


Mastocytosis describes a group of disorders in which pathologic mast cells accumulate in tissues. In the 2016 revisions of the World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues, "mastocytosis" was removed as one of the subtypes under the major category of "myeloproliferative neoplasms" and is now classified as its own major category [1]. In systemic mastocytosis (SM), mast cells infiltrate extracutaneous tissues. Once the diagnosis of SM has been reached, the subtype (variant) of disease must be determined, as treatment and prognosis differ for each disorder.

Topics related to other aspects of mastocytosis are found separately:

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

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

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

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Literature review current through: Dec 2017. | This topic last updated: Mar 10, 2017.
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