临床病例评析——干燥综合征合并血栓性血小板减少性紫癜  

Thrombotic thrombocytopenic purpura in a patient with Sjiigren＇s syndrome and literature review

作　　者：王红江[1] 孔晓丹[1] 邢一达[1] 隋晓燕[1] 张旗[2] 张仲慧[3] 

机构地区：[1]大连医科大学附属第二医院风湿免疫科,116027 [2]大连医科大学附属第二医院血液科,116027 [3]大连医科大学附属第二医院神经内科,116027

出　　处：《中华风湿病学杂志》2012年第10期669-673,共5页Chinese Journal of Rheumatology

摘　　要：目的探讨干燥综合征（SS）多系统损害的临床特点，以便及时、准确诊断，及时治疗，有效减少误诊误治。方法在总结我院诊治1例SS合并血栓性血小板减少性紫癜临床诊治经过的基础上，分析SS合并微血管血栓一出血综合征的临床表现、实验室检查、诊断标准、治疗及相关文献复习。结果SS合并血栓性血小板减少性紫癜典型的临床表现为血小板减少性紫癜、微血管病性溶血性贫血、中枢神经系统症状、肾脏损伤和发热；治疗上首选血浆置换，同时应用糖皮质激素及免疫抑制剂。结论SS合并血栓性血小板减少性紫癜临床表现复杂，容易漏诊、误诊，病情进展快，严重威胁患者的生命，病死率高，需予重视。Objective To investigate the clinical characteristics of multisystem impairment of Sj^gren＇s syndrome （SS） to make quick and accurate diagnose and treatment to decrease the rate of misdiagnosis and mistreatment. Methods A case of thrombotic thromboeytopenic purpura with SS was reported and the related literatures were reviewed. The clinical manifestations, laboratory examinations, diagnostic criteria and therapy of thrombotic thrombocytopenic purpura with SS were analyzed. Results Purpura haemorrhagica, hemolytic anemia, central nervous systemic symptoms, kidney impairment and fever were the representative clinical manifestations of thrombotic thrombocytopenic purpura with SS. Plasmapheresis was the most effective therapy with the background treatment with glucocorticosteroid and immunosuppressive agents. Conclusion Thrombotic thrombocytopenic purpura with SS presents a complicated clinical manifestations, so misdiagnosis and mistreatment is very frequent. More attention should be paid to this specific situation because the disease is rare and has high mortality rate.

关 键 词：干燥综合征 血栓性血小板减少性紫癜 临床 

分 类 号：R554.6[医药卫生—血液循环系统疾病]