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. Author manuscript; available in PMC: 2017 Sep 15.
Published in final edited form as: Cancer Res. 2017 Mar 2;77(6):1261–1270. doi: 10.1158/0008-5472.CAN-16-2234

Table 2.

Updated WHO Classification of Mastocytosis 2016

Cutaneous mastocytosis (CM)

  • Maculopapular CM (MPCM) = urticaria pigmentosa (UP)

  • Diffuse CM (DCM)

  • Mastocytoma of skin

Systemic mastocytosis (SM)

  • Indolent SM (ISM)

  • Smoldering SM (SSM)

  • SM with associated hematologic neoplasm (AHN)*

  • Aggressive SM (ASM)

  • Mast cell leukemia (MCL)

Mast cell sarcoma (MCS)
*

The previous term SM-AHNMD (SM with clonal hematologic non-mast cell-lineage disease) and the new term AHN can be used synonymously.