Dereń-Wagemann Izabela, Kuliszkiewicz-Janus Małgorzata, Kuliczkowski Kazimierz
Klinika Hematologii, Nowotworów Krwi i Transplantacji Szpiku, Samodzielny Publiczny Szpital Kliniczny nr 1 we Wrocławiu.
Postepy Hig Med Dosw (Online). 2009 Nov 26;63:564-76.
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 subvariants of mastocytosis are treated with chemotherapy, targeted therapy, and bone marrow transplantation.
肥大细胞增多症是一组异质性罕见疾病，其特征是肥大细胞在一个或多个器官（如皮肤、骨髓、肝脏、脾脏和淋巴结）中增殖和积聚。根据世界卫生组织的分类，肥大细胞增多症分为七个亚型。症状与介质释放以及肿瘤性肥大细胞浸润导致的器官功能受损有关。发生过敏性休克的风险较高；因此，对患者进行教育非常重要。患者可能无症状。皮肤肥大细胞增多症和惰性系统性肥大细胞增多症采用对症治疗。肥大细胞增多症更具侵袭性的亚型则采用化疗、靶向治疗和骨髓移植进行治疗。
