Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 6 S1 2022 07 06 292 297 Vajihe Marsusi Department of Obstetrics and Gynecology, Faculty of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Ashraf Sadat Jamal Department of Obstetrics and Gynecology, Faculty of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Maasoumeh Saleh Department of Obstetrics and Gynecology, Faculty of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Behnaz Nouri Department of Obstetrics and Gynecology, Faculty of Medicine, Alborz University of Medical Sciences, Karaj, Iran. 2021 02 13 2021 04 14 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). https://crcp.tums.ac.ir/index.php/crcp/article/view/483