Clinical-Pathological Features and Prognosis of Thrombotic Thrombocytopenic Purpura in Patients With Lupus Nephritis
Clinical-Pathological Features and Prognosis of Thrombotic Thrombocytopenic Purpura in Patients With Lupus Nephritis
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DOI：
10.1097/maj.0b013e3181b0c872
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发表时间：
2009-11-01
影响因子：
3.1
通讯作者：
Li Leishi
中科院分区：
文献类型：
--
作者：
Tang Zheng;Luo Chunlei;Li Leishi
背景资料：目的探讨狼疮性肾炎（LN）合并血栓性血小板减少性紫癜（TTP）的临床病理特点及预后。方法：回顾性分析8例LN合并TTP患者的临床病理资料及预后。结果：8例患者临床表现为血小板减少、溶血性贫血、神经系统症状、肾功能损害。6例患者出现发热。8例患者表现为快速进展性肾小球肾炎，1例患者表现为持续性肉眼血尿。8例患者的组织学特征为血栓性微血管病变。所有患者均接受免疫抑制治疗，7例患者接受血液净化治疗。3例病例涉及血浆置换和/或免疫吸附。7例患者接受了中位12个月的随访。死亡1例，腹膜透析3例，未随访1例。随访期间，1例患者能够停止腹膜透析，1例患者改为血液透析。其余3例患者肾功能持续稳定。结论：LN合并TTP患者临床病理改变严重。积极的治疗包括肾脏替代疗法、血浆置换和免疫吸收是有希望的。
Background: We investigated the clinical-pathological features and the prognosis of thrombotic thrombocytopenic purpura (TTP) in patients with lupus nephritis (LN). Methods: A retrospective analysis was performed on the clinical-pathological data and prognosis in 8 patients with LN complicating with TTP. Results: Thrombocytopenia and hemolytic anemia, neurologic symptoms, and renal dysfunction were the clinical manifestations in 8 patients. Six patients had fever. Eight patients presented with rapid progressive glomerulonephritis, and 1 patient with continuous gross hematuria. The histologic features of the 8 patients were thrombotic microangiopathy lesions. Immune-suppressive therapies were administrated in all patients, and blood purification therapy was applied in 7 patients. Three cases involved plasma exchange and/or immunoabsorption. Seven patients received a median follow-up of 12 months. One patient died, 3 cases received peritoneal dialysis, and 1 case failed to follow-tip. During follow-up, 1 case was able to stop peritoneal dialysis, and 1 case changed to hemodialysis. The other 3 patients continued with stable renal function. Conclusion: The patients with LN with TTP have severe clinical-pathological changes. Active treatment including renal replacement therapy, plasma exchange, and immunoabsorption are promising.