侵袭性系统性肥大细胞增生症一例并文献复习

目的 提高对侵袭性系统性肥大细胞增生症（ASM）诊断和治疗的认识.方法 回顾性分析1例ASM患者的临床资料及治疗经过,并复习相关文献.结果 作为系统性肥大细胞增生症（SM）的少见亚型,ASM以多脏器受累为主要临床特点,常伴有骨髓功能障碍、溶骨性损害或肝脾大等表现,提示机体肥大细胞高负荷状态.结论 ASM诊断除了必须符合SM诊断标准外,还须具备1项或多项“C”项所见.ASM患者预后影响因素诸多,准确的预后判断有助于临床治疗策略的制定,治疗应高度个体化.

Aggressive systemic mastocytosis:one case report and literatures review

Objective To improve the acknowledge of diagnosis and therapy of aggressive systemic mastocytosis （ASM）. Methods One ASM patient was reported and the literatures were reviewed. Results As a rare subtype of SM, ASM is characterized by multiple organs involvement, and often accompanied by bone marrow dysfunction, osteolytie lesions and palpable hepatomegaly or splenomegaly which usually indicate the high mast cell burden. Conclusion ASM meets criteria for SM and has one or more ＂C＂ findings. Variable factors affect the prognosis of ASM patients and the formulation of the clinical treatment strategy which leads to the highly individualized therapies.