Therapy and prevention of thrombotic thrombocytopenic purpura during pregnancy: a clinical study of 16 pregnancies.

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder of unknown pathogenesis, with preference to women. The mortality rate of patients with TTP was 90% until the introduction of plasma therapy that increased the survival rate to 70-80%, with minimal or no sequelae. Of the survivors, 30-60% suffer from relapses, often in association with precipitating factors such as infections, surgery, and pregnancy. Women who are either pregnant or in the postpartum period make up 10-25% of TTP patients, and once the disease occurs during a pregnancy, it tends to recur in subsequent ones. We treated five women who suffered at least one TTP episode during pregnancy. They had a total of 16 pregnancies, eight of which were complicated by TTP. They suffered seven additional TTP episodes that were not associated with pregnancy. We assessed the severity of each TTP episode with a scoring system used in our previous studies. Presented is the course of their disease and their pregnancies, and guidelines for the management and prevention of TTP during pregnancy are provided.