Thrombotic Thrombocytopenic Purpura in a Parturient Leading to Life-Threatening Thrombocytopenia and Neonatal Demise—A Case Report

Thrombocytopenia is common (up to 12%) in pregnancy; thrombotic thrombocytopenia is a rare cause of thrombocytopenia; however, it is immediately life-threatening to both mother and fetus and requires immediate intervention. This is compounded by the need to act on a presumptive diagnosis with high level of suspicion given the relatively long laboratory confirmation time. A 31-year-old gravida 3, para 1 parturient at 26 weeks of gestation presented to outside hospital with recent onset of easy bruising and blurry vision. A blood count was drawn and showed a platelet count of 19,000/μL. She was transferred to our institution for management where an initial diagnosis of preeclampsia with severe features was made based on thrombocytopenia and fetal growth restriction (<1%). Platelet count nadired at 14,000/μL and a blood smear showed schistocytes, suggesting microangiopathic hemolysis, prompting discussion of urgent cesarean delivery and anesthesia consult. An urgent hematology consult led to presumptively diagnosing thrombotic thrombocytopenic purpura and cesarean delivery was deferred after discussion with the patient and team. Plasma exchange and steroid therapy were started promptly, and her platelet count improved within 12 hours. Unfortunately, the patient experienced neonatal demise. Undetectable ADAMTS13 levels confirmed diagnosis of thrombotic thrombocytopenia. She was transitioned to rituximab, platelets recovered to baseline, and she continues to do well. Thrombotic thrombocytopenia is a rare, life-threatening cause of thrombocytopenia in pregnancy. Despite grim fetal prognosis, especially in early pregnancy, low threshold of suspicion, early anesthesia involvement, and multi-disciplinary approach can facilitate diagnosis and timely intervention. In our case, it was likely lifesaving.