Navigating Recurrent Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP) in Pregnancy: A Case Report

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal condition characterized by thrombocytopenia and microangiopathic hemolytic anemia. Primarily affecting women of childbearing age, pregnancy is a significant precipitating factor for TTP. The condition's symptoms often overlap with other pregnancy-related disorders, such as pre-eclampsia or HELLP syndrome, making accurate diagnosis particularly challenging. Case Description: A 32-year-old woman, gravida 2 para 1, with a body mass index of 39, presented at 11 weeks gestation with a syncopal episode following a blood draw, along with petechiae, bruising, and thrombocytopenia. Her obstetric history includes a similar clinical presentation during her first pregnancy three years earlier, initially diagnosed as HELLP syndrome, necessitating the emergent delivery of a healthy baby at 34 weeks gestation. However, persistent postpartum thrombocytopenia led to further investigation and a definitive immune TTP (iTTP) diagnosis due to anti-ADAMTS-13 antibodies. She was successfully treated with plasma exchange (PLEX) and high-dose steroids at that time. She remained in remission on low-dose steroid, which eventually was tapered off. In her current pregnancy, she experienced a recurrence of iTTP at 11 weeks GA, notably earlier than her previous episode. Laboratory evaluations showed a platelet count of 41,000/µL, anemia (Hb=8.3 g/dL), elevated lactate dehydrogenase (LDH=400 U/L), normal creatinine, and scant schistocytes on the blood smear. Given her medical history, prednisone and PLEX were initiated. Following three PLEX sessions, her thrombocytopenia and clinical signs resolved, and her platelet count increased to 298,000/µL. The patient was subsequently discharged in a stable condition. Discussion: The incidence and frequency of iTTP increase during pregnancy and can often be misdiagnosed as severe preeclampsia or HELLP syndrome. Plasma-based therapies have significantly improved the prognosis for these patients. Additionally, serial evaluation of ADAMTS-13 activity is recommended both at the preconception stage and throughout pregnancy for women with a history of TTP. This case emphasizes the critical need for heightened awareness and vigilance for TTP relapse during pregnancy.