摘要
目的:探讨系统性红斑狼疮(SLE)相关性血栓性血小板减少性紫癜(TTP)的临床特征、诊断、治疗及转归。方法:回顾性分析河北医科大学第二医院2002年1月至2015年8月收治的12例SLE相关性TTP患者的临床资料。结果:12例SLE相关性TTP患者包括女11例,男1例,中位年龄34.5岁,其中5例为SLE治疗过程中出现的TTP,7例为两者同时诊断;全部患者均有溶血性贫血,血小板减少,神经系统损害,10例有肾脏损害;5例血管性血友病因子裂解蛋白酶(ADAMTS 13)活性均重度减低(<5%),同时伴抑制物阳性;9例外周血涂片破碎红细胞均>1%;12例患者均给予糖皮质激素治疗,11例联合其他药物(10例联合免疫抑制剂,1例联合静脉注射用丙种球蛋白,1例同时加用美罗华);10例给予血浆置换,2例死亡;2例未行血浆置换患者均死亡;4例死亡患者全部伴有肾脏损害。结论:临床表现结合多次血涂片检查有助于SLE相关性TTP的早期诊断,尽早血浆置换联合糖皮质激素是治疗成功的重要措施,肾脏损害可能提示预后不良。
Objective： To investigate the clinical manifestations,treatment strategies and outcomes of 12 patients with systemic lupus erythematosus（ SLE） associated with thrombotic thrombocytopenic purpura（ TTP）. Methods： The clinical data from 12 cases of SLE associated with TTP admitted in the Second Hospital of Hebei Medical University from January 2002 to August 2015 were retrospectively analyzed. Results： 12 cases of SLE associated with TTP included 11 females and 1 male,their median age was 34. 5 years old,among them 5 cases of TTP were diagnosed during the treatment of SLE,7 cases of TTP were comfirmed together with SLE on admission. The hemolytic anemia,thrombocytopenia and neurological deficits appeared in all the patients,the renal impairment was observed in 10 cases,the schistocytes of peripheral blood smears（ 1%） were present in 9 cases,a severely reduction of ADAMTS 13 activity（ 5%） with inhibitor-positive had been demonstrated in 5 cases,all of the 12 patients were treated with glucocorticoid,and 11 cases were treated in combination with other drug（ 10 cases combined with cytotoxics,1 case with intravenous gamma globulin,1 case with rituximab）,plasma exchange were used in 10 cases,and 2 cases died,2 cases without receiving plasma exchange all died,renal damage was observed in all the dead patients. Conclusion： Clinical manifestation and repeated examinations of peripheral blood smears are helpful for early diagnosis of SLE associated with TTP,the plasma exchange combined with glucocortcoids is an effective treatment method,the renal impairment may be a risk factor related with poor prognosis.
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2017年第4期1147-1150,共4页
Journal of Experimental Hematology
