Systemic mastocytosis: Determining the subtype of disease
- Cem Akin, MD, PhD
Cem Akin, MD, PhD
- Associate Professor of Medicine
- Harvard Medical School
- Director, Mastocytosis Center
- Brigham and Women's Hospital
- Jason Gotlib, MD, MS
Jason Gotlib, MD, MS
- Professor of Medicine
- Stanford University School of Medicine
- Section Editor
- Bruce S Bochner, MD
Bruce S Bochner, MD
- Editor-in-Chief — Allergy and Immunology
- Section Editor — Adult Allergy; Asthma
- Samuel M Feinberg Professor of Medicine
- Northwestern University Feinberg School of Medicine
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 . 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:
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- SUBTYPES OF SYSTEMIC MASTOCYTOSIS
- Basic evaluation
- Advanced testing
- Evaluation for organ enlargement or dysfunction
- - B findings
- - C findings
- DETERMINING THE SUBTYPE
- Indolent systemic mastocytosis
- - Isolated bone marrow mastocytosis
- Smoldering systemic mastocytosis
- Systemic mastocytosis with an associated hematologic neoplasm
- Aggressive systemic mastocytosis
- Mast cell leukemia
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS