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Variant – Term (Abbreviation) |
Subvariants |
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• Cutaneous Mastocytosis (CM) |
– Urticaria Pigmentosa (UP) = Maculopapular CM (MPCM) |
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– Diffuse CM (DCM) |
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– Mastocytoma of Skin |
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• Indolent Systemic Mastocytosis (ISM) |
– Smouldering SM (SSM) |
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– Isolated bone marrow mastocytosis (BMM) |
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• Systemic Mastocytosis with an associated clonal hematologic non-mast cell lineage disease (SM-AHNMD) |
– Systemic Mastocytosis with Acute Myeloid Leukemia (SM-AML) |
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– Systemic Mastocytosis with Myelodysplastic Syndrome (SM-MDS) |
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– Systemic Mastocytosis with Myeloproliferative Disorder (SM-MPD) |
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– Systemic Mastocytosis with Chronic Myelomonocytic Leukemia (SM-CMML) |
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– Systemic Mastocytosis with Non-Hodgkin’s Lymphoma (SM-NHL) |
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– Systemic Mastocytosis with Hypereosinophilic Syndrome (SM-HES) |
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• Aggressive Systemic Mastocytosis (ASM) |
– Lymphadenopathic SM with eosinophilia (In a few cases, the FIP1L1/PDGFRA-fusion gene may be detected) |
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• Mast Cell Leucemia (MCL) |
– Aleukemic MCL |
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• Mast Cell Sarcoma (MCS) |
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• Extracutaneous Mastocytoma |
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WHO, World Health Organization
References:
- Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med. 2004;55:419-32. [Medline]
- 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