摘要
目的 探讨系统性红斑狼疮（SLE）合并血栓性血小板减少性紫癜（TTP）的临床特点、诊断及治疗.方法 回顾性分析14例SLE合并TTP者的临床表现、实验室检查结果、诊治和转归.结果 14例SLE合并TTP者中,男4例,女10例,中位年龄23（17 ～ 69）岁.5例以SLE首发,9例两者同时发病.所有患者均有血小板减少、微血管病性溶血性贫血,发热、神经系统异常、肾功能损害的发生比例分别为12/14、11/14、11/14,其中8例患者具有上述五联征.糖皮质激素（单用或联合静脉注射人免疫球蛋白和环磷酰胺）治疗6例,糖皮质激素联合血浆置换8例,有效率分别为2/6、6/8.8例患者存活,6例死亡.对6例存活者进行了随访,均无复发.结论 SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要.SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后.
Objective To analyze the clinical features, diagnosis and treatment of thrombotic thrombocytopenic purpura （TTP） in patients with systemic lupus erythematosus （SLE）. Methods Clinical manifestations, laboratory findings, diagnosis, treatment and prognosis of 14 SLE patients with TTP were retrospectively analyzed. Results Of the 14 patients diagnosed with SLE and TTP, 4 were men and 10 were women. The median age at diagnosis was 23（17-69） years old. In five patients, the onset of SLE preceded TTP, and in nine patients SLE and TIP occurred simultaneously. All the 14 patients had thrombocytopenia and hemolytic anemia, 12 had fever, 11 had neurologic abnormalities, and 11 had renal dysfunction. Eight patients presented with the classic pentad of symptoms. Six patients were given steroids （alone or in combination with intravenous immunoglobulin and cyelophosphamide） , and eight patients were treated with steroids in combination with plasmapheresis, with response rates of 2/6 and 6/8, respectively. Six patients died, with overall mortality rate of 6/14. No patients relapsed during the follow-up period. Conclusions SLE and TrP share some similar clinical symptoms. As a result, repeated examinations of peripheral blood smears are very important for early diagnosis. The renal damage in patients of co-existing diseases is more serious than those with TTP alone or SLE alone. Early diagnosis and prompt treatment with plasma exchange and steroids may improve the prognosis in SLE patients with TIP.
出处
《中华内科杂志》
CAS
CSCD
北大核心
2013年第5期383-386,共4页
Chinese Journal of Internal Medicine
关键词
红斑狼疮
系统性
紫癜
血栓性血小板减少性
疾病特征
治疗
Lupus erythematosus, systemic
Purpura, thrombotic thrombocytopenic
Disease attributes
Treatment
