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TTP- not short for Time To Panic: A Multidisciplinary Approach to Managing Thrombotic Thrombocytopenic Purpura in Pregnancy
Abstract Number: RF4AD-442
Abstract Type: Case Report Case Series
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia, and varying degrees of end-organ dysfunction. When present in pregnancy, it can mimic other conditions in the general population, such as hemolytic uremic syndrome, and those unique to the puerpartum, such as preeclampsia. Rapid and appropriate diagnosis is important, as delay in care can be life-threatening. We present the case of a multigravida with a rare hereditary form of TTP, Upshaw Schulman syndrome. Her pregnancy was further complicated by HELLP syndrome and an emergent cesarean delivery illustrating the importance of a multidisciplinary team in the timely management of an acutely complicated patient.
39 yo G11.P184.108.40.206 at 25.3 wks EGA with a history of thrombotic thrombocytopenic purpura (TTP) complicating two prior pregnancies, pulmonary embolism, and chronic hypertension. At the time of her initial presentation, she had a platelet count of 7,000/mm3 and ADAMSTS13 of 9% with a positive inhibitor screening suggestive of hereditary TTP or Upshaw Shulman syndrome Physical exam was notable for diffuse petechiae, altered mental status, and tea-colored urine. She was subsequently admitted to the intensive care unit for daily plasma exchange, and responded to 7 rounds of plasma exchange, after which her platelets improved to 183,000 giving her sufficient time for magnesium sulfate and betamethasone to be administered. At 26.6 wks EGA, however, platelet count started dropping to 20,000 and was refractory to 9 additional rounds of plasmapheresis, raising concern for HELLP. Rituximab could not be used intrapartum due to increased risk of neonatal B cell lymphoma and an emergency cesarean section was called for thrombocytopenia unresponsive to treatment. General anesthesia was administered using a rapid sequence induction. Intubation attempts were traumatic due to upper airway edema and poor glottis visualization. Nitrous oxide was used for maintenance to minimize the dose of halogenated agents and Pitocin and Hemabate were all given prophylactically to decrease risk of uterine atony and postpartum hemorrhage. The patient remained intubated for two days until upper airway edema subsided. To minimize bleeding risk, the patient was transfused with 2 units of platelets and 2 units of pRBCs due to a preoperative Hgb of 6.6. Postpartum, her platelet count improved to 254,000/mm^3 after receiving 13 additional plasmapheresis treatments, prednisone, and solumedrol.
Conclusion: While the diagnosis of Upshaw Schulman is rare, emergency management is essential in the proper care of parturients presenting with platelet disorders and thrombocytopenia. When recognized a multidisciplinary approach of obstetricians, obstetric anesthesiologists, maternal-fetal medicine specialists, hematologists, and neonatologists is necessitated for safe and timely care.