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Cyclosporine-associated Thrombotic Microangiopathy and Thrombocytopenia- associated Multiple Organ Failure: A Case Successfully Treated With Therapeutic Plasma Exchange

发布于：2013年4月29日    文字：【大】【中】【小】

J Pediatr Hematol Oncol. 2013 Apr 24. [Epub ahead of print]

Odek C, Kendirli T, Yaman A, Ileri T, Kulo?lu Z, Ince E.

Abstract:

Thrombotic microangiopathy (TMA) is characterized by microvascular thrombosis, thrombocytopenia, and microangiopathic hemolytic anemia. Previous studies have shown that cyclosporine (CsA) is associated with TMA but the number of reported cases is very limited. We describe a 13-year-old girl with CsA-associated TMA and thrombocytopenia-associated multiple organ failure (TAMOF). CASE REPORT:: The patient was diagnosed with polyglandular deficiency syndrome and had a history of celiac disease, autoimmune thyroiditis, and diabetes mellitus typeI.CsA was started 7 months before her admission to our pediatric intensive care unit for persistent diarrhea associated with celiac disease. At the time of her admission to our pediatric intensive care unit, she was thrombocytopenic and anemic with multiple organ failure. Laboratory and clinical findings were consistent with TMA and TAMOF. CsA was discontinued and therapeutic plasma exchange was performed daily for 5 days. The patient improved clinically, laboratory findings normalized, and TMA and multiple organ failure dissolved. CONCLUSION:: This case report indicates that therapeutic plasma exchange may be effective in the treatment of CsA-associated TMA and TAMOF, especially in the presence of systemic findings

摘要：

环孢素A可以引起血栓性微血管病，其机制与环孢素A直接引起内皮细胞损伤相关，同时环孢素A可以增加血小板聚集，也有个别报道提出与补体旁路活化相关。环孢素A引起的血栓性微血管病病理改变基本上都局限在肾脏。通常停用环孢素可以使病变逆转。本报道了一例13岁多腺体缺陷综合症女孩，其存在腹腔内病变、自身免疫性甲状腺炎、1型糖尿病，患者使用7个月环孢素A后出现贫血、血小板减少、多器官功能衰竭，实验室检查符合血栓性微血管病，予停用环保素A及每日血浆置换共5次，患者贫血、血小板减少和器官功能衰竭基本缓解。结论，对于环孢素A相关的血栓性微血管病、特别是伴有全身表现的多器官功能损害者可以考虑使用血浆置换。

http://www.ncbi.nlm.nih.gov/pubmed/23619107 [Abstract]