An Overview of Clinical and Physiopathological Features of Thrombotic Thrombocytopenic Purpura During Pregnancy

Thrombotic thrombocytopenic purpura (TTP), haemolytic-uraemic syndrome (HUS), preeclampsia- HELLP (haemolysis, elevated liver enzymes, low platelet count) syndrome, and some other autoimmune syndromes like catastrophic antiphospholipid syndrome (CAPS), are microangiopathic disorders that can be diagnosed during pregnancy. Although the underlying physiopathological mechanisms differ, the clinical consequences are very similar in all of them, so that it is very difficult to establish a differential diagnosis. Since each disease has its own treatment particularities, and maternal and perinatal morbidity and mortality are high when treatment is not appropriate, gynaecologists need to have a thorough understanding of differentiating characteristics of these disorders. TTP is more common in women, with a peak incidence in the fourth decade of life, and 10% of all cases occur during pregnancy. In the absence of adequate diagnosis and treatment, the maternal and foetal mortality rate approaches 90%. Preconceptional counselling should be provided to women with prior episodes of TTP or congenial Upshaw-Schülman syndrome.