Salameh Hassan, Abu Omar Mohannad, Alhariri Ahmad, Kisra Sood, Qasem Abdulraheem, Abdulhak Aref Bin
1Department of Medicine, School of Medicine, University of Missouri-Kansas City, Kansas City, MO; and 2Department of Pediatrics, Division of Medical Genetics, University of California San Francisco Medical Center, San Francisco, CA.
Am J Ther. 2016 Jul-Aug;23(4):e1110-5. doi: 10.1097/MJT.0000000000000133.
Hemolytic uremic syndrome is the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. When not associated with enteric infection, it is classified as atypical hemolytic uremic syndrome (aHUS) and carries a worse outcome with high mortality rate and up to 50% of the survivors will end up with end-stage renal disease. Renal transplant was restricted to a very small percentage of patients due to high recurrence rate posttransplant that approaches 90%. Our case describes a posttransplant adult patient with familial aHUS whom was successfully treated with eculizumab. We also reviewed all other reported cases of adult posttransplant aHUS, both familial and sporadic, which were treated with eculizumab. In summary, eculizumab might expand the utility of renal transplant for patients with end-stage renal disease due to aHUS.
溶血性尿毒症综合征是由非免疫性微血管病性溶血性贫血、血小板减少症和急性肾衰竭组成的三联征。当与肠道感染无关时,它被归类为非典型溶血性尿毒症综合征(aHUS),其预后较差,死亡率高,高达50%的幸存者最终会发展为终末期肾病。由于移植后复发率高达90%,肾移植仅限于极少数患者。我们的病例描述了一名患有家族性aHUS的移植后成年患者,他接受依库珠单抗治疗成功。我们还回顾了所有其他报道的成年移植后aHUS病例,包括家族性和散发性病例,这些病例均接受了依库珠单抗治疗。总之,依库珠单抗可能会扩大肾移植对因aHUS导致终末期肾病患者的应用范围。