THE LONG-TERM CONSEQUENCES OF THROMBOTIC MICROANGIOPATHY (THROMBOTIC THROMBOCYTOPENIC PURPURA AND HEMOLYTIC-UREMIC SYNDROME) IN PREGNANCY

Objective: To characterize perinatal outcomes and long-term maternal c omplications from thrombotic microangiopathy manifested during pregnan cy, and to review the clinical course and long-term follow-up of pregn ant women with this condition at our institution over the past 25 year s. Methods: We identified prospectively pregnant women who met clinica l and laboratory criteria for thrombotic thrombocytopenic purpura or h emolytic uremic syndrome. Their clinical and laboratory findings, resp onse to treatment, perinatal outcomes, and long-term sequelae were the n analyzed. Results: Between 1972 and 1997, 11 women had 13 pregnancie s complicated by thrombotic microangiopathy, representing an incidence of one per 25,000 births. In three pregnancies (23%), severe and refr actory disease developed before midpregnancy. In ten other pregnancies , disease developed either peripartum (62%) or several weeks postpartu m (15%). In only two pregnancies with peripartum or postpartum onset o f disease was there a clinical picture of severe preeclampsia. In gene ral, the response to treatment was prompt. One woman died of her initi al disease in early pregnancy, and mean follow-up of nine survivors wa s 8.7 years. Disease recurred at least once in 50% of these, two durin g a subsequent pregnancy. There was at least one serious long-term seq uela in all but two survivors; these included recurrence of thrombotic microangiopathy, renal failure, severe hypertension, chronic blood-bo rne infections, and death. Conclusion: Thrombotic microangiopathy comp licating pregnancy is rare, and with careful evaluation, it should not be confused with atypical preeclampsia. With prompt and aggressive tr eatment including plasma exchange, the likelihood of immediate surviva l is high; however, long-term morbidity and mortality are common. (C) 1998 by The American College of Obstetricians and Gynecologists.