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WHO Classification of Mastocytosis

Variant – Term (Abbreviation)


• Cutaneous Mastocytosis (CM)

– Urticaria Pigmentosa (UP) = Maculopapular CM (MPCM)

– Diffuse CM (DCM)

– Mastocytoma of Skin

• Indolent Systemic Mastocytosis (ISM)

– Smouldering SM (SSM)

– Isolated bone marrow mastocytosis (BMM)

• Systemic Mastocytosis with an associated clonal hematologic non-mast cell lineage disease (SM-AHNMD)

– Systemic Mastocytosis with Acute Myeloid Leukemia  (SM-AML)

– Systemic Mastocytosis with Myelodysplastic Syndrome (SM-MDS)

– Systemic Mastocytosis with Myeloproliferative Disorder (SM-MPD)

– Systemic Mastocytosis with Chronic Myelomonocytic Leukemia (SM-CMML)

– Systemic Mastocytosis with Non-Hodgkin’s Lymphoma (SM-NHL)

– Systemic Mastocytosis with Hypereosinophilic Syndrome (SM-HES)

• Aggressive Systemic Mastocytosis (ASM)

– Lymphadenopathic SM with eosinophilia (In a few cases, the FIP1L1/PDGFRA-fusion gene may be detected)

• Mast Cell Leucemia (MCL)

– Aleukemic MCL

• Mast Cell Sarcoma (MCS)


• Extracutaneous Mastocytoma


WHO, World Health Organization

Related Criteria

Diagnostic Criteria for Systemic Mastocytosis (SM)

Classification of Neutropenia

Classification of Neutrophilia

Serum Levels That Differentiate Anemia of Chronic Disease from Iron-Deficiency Anemia

Diagnostic Criteria for Disseminated Intravascular Coagulation (DIC)

Clinical Conditions Associated with Disseminated Intravascular Coagulation (DIC)

Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome (HES)

FAB Classification of Myelodysplastic Syndromes (MDS)

Diagnostic Criteria for Heparin-Induced Thrombocytopenia (HIT)




  1. Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med. 2004;55:419-32. [Medline]
  2. O'Brien S, Tefferi A, Valent P. Chronic myelogenous leukemia and myeloproliferative disease. Hematology (Am Soc Hematol Educ Program). 2004;:146-62. [Medline]

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Created: June 21, 2005
Last Modified: 10/23/2010

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