Eculizumab treatment of thrombotic microangiopathy in a patient with ulcerative colitis

Secondary thrombotic microangiopathy (TMA) can be induced by several underlying conditions and drugs, yet coexistence of TMA and inflammatory bowel disease (IBD) has only infrequently been documented. A successful management beyond supportive care in cases with secondary TMA represents a challenge, as some underlying conditions might amplify complement dysregulation or even unmask a genetic predisposition to atypical hemolytic uremic syndrome (HUS)-both of which could require treatment with a complement blocking agent. We observed a case in which TMA developed in a patient with ulcerative colitis (UC). Genetic screening showed a heterozygous mutation in diacylglycerol kinase ε (DGKE). Eculizumab resulted in complete resolution of TMA, however UC relapsed after cessation of eculizumab.