Postpartum thrombotic thrombocytopenic purpura complicated by Budd-Chiari syndrome

Thrombotic thrombocytopenic purpura is an infrequent but devastating complication of pregnancy, often difficult to differentiate from severe preeclampsia and the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). To our knowledge, the combination of thrombotic thrombocytopenic purpura and hepatic vein thrombosis has not been reported previously in pregnancy.

A 33-year-old woman, a multigravida, was delivered at 36 weeks' gestation because of pregnancy-induced hypertension and HELLP syndrome. Postpartum, the patient became obtunded, disoriented, and anuric. Her laboratory values revealed a Coombs-negative, microangiopathic hemolytic anemia, modestly abnormal coagulation studies, and thrombocytopenia. She also had elevated liver enzymes, lactate dehydrogenase, and creatinine. The largest plasma von Willebrand factor multimeric forms were relatively decreased in her ethylenediaminetetra-acetic acid-platelet-poor plasma. A diagnosis of thrombotic thrombocytopenic purpura was made. The patient received plasma exchange, hemodialysis, blood transfusion, and glucocorticoids. 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 demonstrated the absence of flow in the middle and left hepatic veins, secondary to thrombosis (Budd-Chiari syndrome). She was maintained on warfarin therapy and was discharged on postpartum day 50.

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 lifesaving.