WHO classification of mastocytosis.*
| Variant - Term . | Abbreviation . | Subvariants . |
|---|---|---|
| * For details of the WHO classification of mastocytosis see refs #13 and #14. | ||
| ** In a few cases, the FIP1L1/PDGFRA-fusion gene may be detected | ||
| Abbreviations: WHO, World Health Organization; CM, cutaneous mastocytosis; SM, systemic mastocytosis; AML, acute myeloid leukemia; MDS, myelodysplastic syndrome; MPD, myeloproliferative disorder; CMML, chronic myelomonocytic leukemia; MCL, mast cell leukemia; NHL, non-Hodgkin’s lymphoma. | ||
| • 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 | – SM-AML – SM-MDS – SM-MPD – SM-CMML – SM-NHL – SM-HES |
| • Aggressive Systemic Mastocytosis | ASM | – Lymphadenopathic SM with eosinophilia** |
| • Mast Cell Leukemia | MCL | – Aleukemic MCL |
| • Mast Cell Sarcoma | MCS | |
| • Extracutaneous Mastocytoma | ||
| Variant - Term . | Abbreviation . | Subvariants . |
|---|---|---|
| * For details of the WHO classification of mastocytosis see refs #13 and #14. | ||
| ** In a few cases, the FIP1L1/PDGFRA-fusion gene may be detected | ||
| Abbreviations: WHO, World Health Organization; CM, cutaneous mastocytosis; SM, systemic mastocytosis; AML, acute myeloid leukemia; MDS, myelodysplastic syndrome; MPD, myeloproliferative disorder; CMML, chronic myelomonocytic leukemia; MCL, mast cell leukemia; NHL, non-Hodgkin’s lymphoma. | ||
| • 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 | – SM-AML – SM-MDS – SM-MPD – SM-CMML – SM-NHL – SM-HES |
| • Aggressive Systemic Mastocytosis | ASM | – Lymphadenopathic SM with eosinophilia** |
| • Mast Cell Leukemia | MCL | – Aleukemic MCL |
| • Mast Cell Sarcoma | MCS | |
| • Extracutaneous Mastocytoma | ||