Unifying Concepts

WHO Classification of Mastocytosis

Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor. The symptoms of systemic mastocytosis are due to the pathologic accumulation and activation of mast cells in various tissues such as bone marrow, skin, gastrointestinal tract, liver, and spleen.

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: Jun 21, 2005

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