The classification of systemic mastocytosis should include mast cell leukemia (MCL) and systemic mastocytosis with a clonal hematologic non-mast cell lineage disease (SM-AHNMD).

摘要

We have read with interest the letter of Drs Pardanani and Tefferi, suggesting that the current World Health Organization (WHO) classification of systemic mastocytosis (SM) should be revised. They suggest eliminating mast cell leukemia (MCL) and SM with associated clonal hematologic non-mast cell lineage disorder (SM-AHNMD) as variants, and to regard smoldering SM (SSM) as a separate category. We first note that the EU/US Consensus-Group defined criteria for SSM in 2001 and proposed in 2007 that SSM should be a separate category of SM. In 2001, SSM was defined as a provisional subcategory of indolent SM (ISM), a concept adopted by the WHO in 2001 and preserved in 2008.