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移植相关血栓性微血管病:静脉注射免疫球蛋白作为初始治疗的作用

Transplant-associated thrombotic microangiopathy: the role of IgG administration as initial therapy.

作者信息

Hochstetler L A, Flanigan M J, Lager D J

机构信息

Department of Internal Medicine, University of Iowa, Iowa City.

出版信息

Am J Kidney Dis. 1994 Mar;23(3):444-50. doi: 10.1016/s0272-6386(12)81010-9.

DOI:10.1016/s0272-6386(12)81010-9
PMID:8128949
Abstract

Two transplant patients, one a renal-pancreas and the other a liver allograft recipient, are reported. Both developed clinical and histologic evidence of cyclosporine-associated thrombotic microangiopathy and responded favorably to intravenous IgG therapy. An additional 20 cadaveric renal transplant recipients who developed cyclosporine-associated thrombotic microangiopathy are reviewed. The clinical and laboratory presentation of posttransplant thrombotic microangiopathy varied. Elevated serum creatinine and lactic dehydrogenase (LDH) levels were the most consistent, albeit nonspecific, findings at diagnosis. Fourteen of 22 patients (64%) presented with thrombocytopenia and 19 (86%) had a hemolytic anemia verified on diagnosis. Histologic evidence of thrombotic microangiopathy was present in renal biopsies from each of the renal allograft recipients and a skin biopsy from the liver allograft recipient. Treatment included withdrawal or reduction of the cyclosporine dose, plasmapheresis, or administration of intravenous IgG. There was an overall renal allograft loss of 57%, which included five deaths. Symptomatic cytomegalovirus infection was more common than expected in this patient group (P = 0.038) and may, in combination with cyclosporine therapy, have predisposed these patients to develop clinically significant thrombotic microangiopathy. Transplant-associated microangiopathy appears to be a relatively common disorder associated with a substantial increase in early graft loss (P = 0.005) and mortality (P = 0.001).

摘要

报告了两名移植患者,一名是肾胰腺移植受者,另一名是肝脏移植受者。两人均出现了与环孢素相关的血栓性微血管病的临床和组织学证据,并对静脉注射免疫球蛋白治疗反应良好。另外对20名发生与环孢素相关的血栓性微血管病的尸体肾移植受者进行了回顾。移植后血栓性微血管病的临床和实验室表现各不相同。血清肌酐和乳酸脱氢酶(LDH)水平升高是诊断时最一致的发现,尽管是非特异性的。22名患者中有14名(64%)出现血小板减少,19名(86%)在诊断时证实有溶血性贫血。血栓性微血管病的组织学证据存在于每位肾移植受者的肾活检以及肝脏移植受者的皮肤活检中。治疗措施包括停用或减少环孢素剂量、血浆置换或静脉注射免疫球蛋白。总体肾移植丢失率为57%,其中包括5例死亡。有症状的巨细胞病毒感染在该患者组中比预期更常见(P = 0.038),并且可能与环孢素治疗一起使这些患者易发生具有临床意义的血栓性微血管病。移植相关的微血管病似乎是一种相对常见的疾病,与早期移植物丢失(P = 0.005)和死亡率(P = 0.001)大幅增加相关。

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