maternal death due to refractory thrombotic thrombocytopenic purpura (ttp) associated with superimposed preeclampsia, sepsis, and intracranial hemorrhage: a case report

Thrombotic thrombocytopenic purpura (ttp) is an acute, rare, potentially life-threatening disorder presenting with thrombocytopenia, hemolytic anemia, and clinical consequences of microvascular thrombosis caused by a deficiency of adams13 [1, 2]. the incidence of acquired ttp is approximately three cases per one million adults per year, based on the oklahoma ttp-hemolytic uremic syndrome (hus) registry [3]. here, we present a case of ttp with persistent severely deficient adams13 activity and recurrent relapses. she had a refractory relapse in the third trimester of pregnancy, complicated with superimposed severe preeclampsia and sepsis, and finally expired due to intracranial hemorrhage (ich).