POSTPARTUM THROMBOTIC THROMBOCYTOPENIC PURPURA COMPLICATED BY BUDD-CHIARI SYNDROME

Background: Thrombotic thrombocytopenic purpura is an infrequent but d evastating complication of pregnancy, often difficult to differentiate from severe preeclampsia and the syndrome of hemolysis, elevated live r enzymes, and low platelets (HELLP). To our knowledge, the combinatio n of thrombotic thrombocytopenic purpura and hepatic vein thrombosis h as not been reported previously in pregnancy. Case: A 33-year-old woma n, a multigravida, was delivered at 36 weeks' gestation because of pre gnancy-induced hypertension and HELLP syndrome. Postpartum, the patien t became obtunded, disoriented, and anuric. Her laboratory values reve aled a Coombs-negative, microangiopathic hemolytic anemia, modestly ab normal coagulation studies, and thrombocytopenia. She also had elevate d liver enzymes, lactate dehydrogenase, and creatinine. The largest pl asma von Willebrand factor multimeric forms were relatively decreased in her ethylenediaminetetra-acetic acid-platelet-poor plasma. A diagno sis of thrombotic thrombocytopenic purpura was made. The patient recei ved plasma exchange, hemodialysis, blood transfusion, and glucocortico ids. She responded to therapy, but was later noted to have increasing hepatosplenomegaly, rising levels of bilirubin, and elevated alkaline phosphatase. A Doppler study and magnetic resonance imaging demonstrat ed the absence of now in the middle and left hepatic veins, secondary to thrombosis (Budd-Chiari syndrome). She was maintained on warfarin t herapy and was discharged on postpartum day 50. Conclusion: The early recognition of thrombotic thrombocytopenic purpura is crucial for the initiation of appropriate treatment as soon as possible. The avoidance of platelet transfusion and early plasma-exchange therapy may be life saving.