THROMBOTIC THROMBOCYTOPENIC PURPURA AND HEMOLYTIC-UREMIC SYNDROME IN PREGNANCY - REVIEW OF 11 CASES

OBJECTIVE: Little information exists regarding thrombotic thrombocytop enic purpura and hemolytic uremic syndrome during pregnancy. We report a series of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome complicating pregnancy, with emphasis on diagnosis and manage ment of this rare disorder. STUDY DESIGN: Between January 1988, and Fe bruary 1996, 11 women with either thrombotic thrombocytopenic purpura (n = 8) or hemolytic uremic syndrome (n = 3) were evaluated. Clinical and laboratory findings and maternal and neonatal outcomes were record ed from the medical records. RESULTS: Eight of the 11 women were in th e third trimester or peripartum period, and three were seen before fet al viability. Treatment included fresh-frozen plasma in all women, pla smapheresis (n = 8), packed red blood cells (n = 9), and platelet tran sfusions (n = 5); 1 patient required splenectomy. There were two mater nal deaths. Of the 3 surviving women, 4 had chronic renal disease, 1 o f whom also had residual neurologic deficit. Preterm delivery occurred in 5 of 8 pregnancies continuing beyond 20 weeks. Indications for del ivery in these 5 women included worsening maternal medical disease, no nreassuring fetal testing, and spontaneous preterm labor. Six of 8 wom en with viable fetuses underwent cesarean delivery. These 6 infants we re born in good condition without thromhocytopenia. Of the remaining 2 infants delivered vaginally, one was healthy at 35 weeks and the othe r was stillborn. CONCLUSION: Thrombotic thrombocytopenic purpura and h emolytic uremic syndrome complicating pregnancy is associated with hig h maternal mortality and long-term morbidity. Preterm delivery and int rauterine fetal death are frequent complications of these pregnancies. Improved survival after this disorder has been attributed to aggressi ve treatment with plasma transfusion or plasmapheresis.