[Pregnancy-associated thrombotic microangiopathy--a diagnostic and therapeutic challenge]

Thrombotic microangiopathies are diseases rarely associated with pregnancy. The pathogenesis might be related to severe preeclampsia and HELLP syndrome.

In May 2000, we saw a 26-year-old primigravida in the 39th gestational week with worsening anemia, thrombocytopenia, and increasing liver enzymes. The diagnosis of HELLP syndrome was made and delivery initiated. Postpartum liver function stabilized, but anemia, thrombocytopenia, and preexisting hypertension worsened. Additionally, renal function deteriorated, and she had to be dialyzed 12 days after delivery. Renal biopsies were performed on day 12, 34, and 60 after delivery. The material showed a thrombotic microangiopathy, initially in an active stage. Later, fibrosis of the preglomerular arterioles and the glomeruli as well as progressive tubulointerstitial damage could be shown. Plasmapheresis was started; substitution was performed with fresh frozen plasma (FFP). Simultaneously, the patient was treated with corticosteroids. After 24 days, we began with cyclophosphamide pulses. Overall, 28 plasmapheresis sessions and three cyclophosphamide pulses were given. In spite of this aggressive regimen, renal function did not recompensate, and renal replacement therapy with continuous ambulatory peritoneal dialysis (CAPD) was initiated.

This course shows that mortality could be decreased using plasmapheresis therapy, but further research is needed to introduce more specific, kidney-protective regimens.