自身免疫性血细胞减少症合并血栓形成7例临床分析

Clinical analysis of autoimmunic hemocytopenia complicated with thrombosis.

目的探讨自身免疫性血细胞减少症患者合并血栓形成的临床特点及其可能机制。方法分析天津医科大学总医院2007—2008年住院诊治的7例自身免疫性血细胞减少症合并血栓形成患者的临床及实验室资料。7例自身免疫性血细胞减少症患者1例为Evans综合征、6例为免疫相关性血细胞减少症。合并下肢静脉栓塞3例、脑血管及肺动脉栓塞各2例、脾动脉及冠状动脉栓塞各1例,其中3例患者同时并发2个部位血管栓塞。6例D-二聚体升高。结果予糖皮质激素、环孢素、静脉丙种球蛋白等药物治疗自身免疫性血细胞减少;予阿司匹林、肝素、华法林等抗栓治疗。治疗2周,血栓栓塞症状消失,治疗1个月,复查血管彩超或CT,栓塞的程度和范围较前明显好转,凝血功能部分或全部恢复正常。目前除1例患者死于严重肺感染外,其余6例患者均未复发。结论血栓栓塞是自身免疫性疾病的一个少见但严重的合并症,及时抗栓及免疫抑制治疗可获理想疗效。

Objective To probe the clinical features and possible mechanisms of autoimmunic hemocytopenia complicated with thrombosis. Methods From 2007 to 2008, the clinical and laboratory data of seven patients with autoimmunic hemocytopenia complicated with thrombosis in the hematology department General Hospital tianjin medical university were analysed. Results One of the patients was with Evans syndrome, and another six patients with immuno-related pancytopenia. Three were complicated with lower extremity Veins embolism;two with cerebral vessel and pulmonary artery embolisms respectively; one with splenic artery and coronary artery embolisms respectively. Among them, three were complicated embolism of two parts;six patients had higher D-Dimer level. They were treated with glucocortieoids, cyclosporin A, and immunoglobulin for immunic hemocytopenia and with anti-coagnlation therapy （ aspirin.heprin and warfarin） for thrombosis. Seven patients＇ hemogram, signs and symptoms improved gradually since they received immunosuppression and anticoagulation therapy. The symptoms of thrombosis disappeared two weeks later. The degree and extent of thrombosis also alleviated;at the same time,the function of blood coagulation recovered partly or completely. Conclusion Thrombosis is a rare but severe complication of autoimmunic hemoeytopenia. Promptly anti-coagulation and immunosuppressive therapies can get ideal therapeutic effects.