Unifying Concepts

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



  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]

Created: June 21, 2005

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