One case of systemic mastocytosis with associated clonal hematological non-mast-cell lineage disease and literatures revivew / 白血病·淋巴瘤

ABSTRACT

Objective To improve the understanding of systemic mastocytosis(SM) with associated clonal haematological non-mast-cell lineage disease (SM-AHNMD). Methods The clinical and laboratory features, treatment and prognosis of a rare case of SM-AHNMD was reported and the related literatures were reviewed. Results As the second most common subtype of SM, SM-AHNMD mainly has multifocal dense infiltrates of mast cells detected in sections of bone marrow and/or other extracutaneous organs, and some special cytology or biochemical markers(toluidine blue staining,tryptase,CD117,CD25 and/or CD2) results are positive. C-kit mutations are found in most adult patients. SM-AHNMD also has clinical characters of other clonal haematological non-mast-cell lineage disease (HNMD), such as myelodysplastic syndrome,myeloproliferative neoplasms, acute myeloid leukemia. Conclusion SM-AHNMD meets criteria for SM and other HNMD. Treatment for SM-AHNMD is mainly about the therapy of HNMD, and the prognosis greatly depends on the accompanying hematological disorder.There is no special treatment for SM,and the treatment should be directed at symptomatic control.