Новые критерии диагностики лейкемического системного мастоцитоза и миеломастоцитозной лейкемии

Abstract

Mast cell leukemia is characterized by proliferation and accumulation of immature mast cells in the bone marrow and other organs. The difficulties exist in the differential diagnosis of leukemic systemic mastocytosis and myelomastocytic leukemia. Although in both cases diagnostic criteria are reported, some questions about the terms remain to be open. This problem was discussed by the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) in 2011--2013. The diagnosis of myelomastocytic leukemia as myeloid tumor with a large number of mast cells was proposed as eligible in the absence of diagnostic criteria of mastocytosis. It was also recommended to divide leukemic systemic mastocytosis into acute and chronic forms based on the presence or absence of cutaneous manifestations. The primary form of the mast cell leukemia must be differentiated from the secondary, which usually develops in the set of aggressive systemic mastocytosis and mast cell sarcoma. The authors put emphasis on the inevitability of the prephase or leukemic systemic mastocytosis, which is often debut as aggressive systemic mastocytosis with rapid progression and the appearance of from 5 to 19% of mast cells in bone marrow smears. This condition is recommended to call the aggressive systemic mastocytosis in conversion to mast cells leukemia. The expansion of the current WHO classification by incorporating different variants of mast cell leukemia, will optimize the selection of patients for clinical trials.