WHO classification of mastocytosis
| Variant . | Subvariants . |
|---|---|
| Cutaneous mastocytosis | Urticaria pigmentosa |
| Maculopapular | |
| Diffuse | |
| Solitary mastocytoma of skin | |
| Indolent systemic mastocytosis | Smoldering SM |
| Isolated bone marrow mastocytosis | |
| Systemic mastocytosis with an associated clonal hematologic non–MC-lineage disease | SM-AML |
| SM-MDS | |
| SM-MPD | |
| SM-CEL or SM-HES | |
| SM-CMML | |
| SM-NHL | |
| Aggressive systemic mastocytosis | |
| MC leukemia | |
| MC sarcoma | |
| Extracutaneous mastocytom |
| Variant . | Subvariants . |
|---|---|
| Cutaneous mastocytosis | Urticaria pigmentosa |
| Maculopapular | |
| Diffuse | |
| Solitary mastocytoma of skin | |
| Indolent systemic mastocytosis | Smoldering SM |
| Isolated bone marrow mastocytosis | |
| Systemic mastocytosis with an associated clonal hematologic non–MC-lineage disease | SM-AML |
| SM-MDS | |
| SM-MPD | |
| SM-CEL or SM-HES | |
| SM-CMML | |
| SM-NHL | |
| Aggressive systemic mastocytosis | |
| MC leukemia | |
| MC sarcoma | |
| Extracutaneous mastocytom |
Adapted from Swerdlow et al.1
MC indicates mast cell; SM, systemic mastocytosis; AML, acute myeloid leukemia; MDS, myelodysplastic syndrome; MPN, myeloproliferative neoplasia; CEL, chronic eosinophilic leukemia; HES, hypereosinophilic syndrome; CMML, chronic myelomonocytic leukemia; and NHL, non-Hodgkin lymphoma.