Mastocytosis - diagnostic criteria and treatment

作者:Deren Wagemann Izabela*; Kuliszkiewicz Janus Malgorzata; Kuliczkowski Kazimierz
来源:Postepy Higieny i Medycyny Doswiadczalnej, 2009, 63: 564-576.

摘要

Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The symptoms are associated with mediator release and impaired organ function due to infiltration by neoplastic mast cells. There is a higher risk of anaphylactic shock; therefore education of the patients is very important. Patients may be asymptomatic. Symptomatic treatment is used in cutaneous mastocytosis and in indolent systemic mastocytosis. More aggressive sub subvariants of mastocytosis are treated with chemotherapy, targeted therapy, and bone marrow trans transplantation.

  • 出版日期2009-11-26