Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in pregnancy: review of 11 cases.

OBJECTIVE

Little information exists regarding thrombotic thrombocytopenic 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 management of this rare disorder.

STUDY DESIGN

Between January 1988 and February 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 recorded from the medical records.

RESULTS

Eight of the 11 women were in the third trimester or peripartum period, and three were seen before fetal viability. Treatment included fresh-frozen plasma in all women, plasmapheresis (n = 8), packed red blood cells (n = 9), and platelet transfusions (n = 5); 1 patient required splenectomy. There were two maternal deaths. Of the 9 surviving women, 4 had chronic renal disease, 1 of whom also had residual neurologic deficit. Preterm delivery occurred in 5 of 8 pregnancies continuing beyond 20 weeks. Indications for delivery in these 5 women included worsening maternal medical disease, nonreassuring fetal testing, and spontaneous preterm labor. Six of 8 women with viable fetuses underwent cesarean delivery. These 6 infants were born in good condition without thrombocytopenia. Of the remaining 2 infants delivered vaginally, one was healthy at 35 weeks and the other was stillborn.

CONCLUSION

Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome complicating pregnancy is associated with high maternal mortality and long-term morbidity. Preterm delivery and intrauterine fetal death are frequent complications of these pregnancies. Improved survival after this disorder has been attributed to aggressive treatment with plasma transfusion or plasmapheresis.