Thrombotic thrombocytopenic purpura in pregnancy

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder with unknown pathophysiology and pathogenesis that is characterized by the classic pentad of thrombocytopenia, Coombs-negative microangiopathic hemolytic anemia, fever, fluctuating neurologic abnormalities, and renal dysfunction. TTP is uncommon and is associated with high maternal and fetal morbidity and mortality. Initial symptoms of TTP may be confused with severe preeclampsia and the HELLP syndrome; therefore, the clinician must maintain a high index of suspicion for the disease. Plasma exchange with fresh frozen plasma is associated with an increased survival rate and is now the treatment of choice. In pregnant women with TTP, delivery of the fetus does not ameliorate the maternal disease process. After initial treatment and remission, patients with a diagnosis of TTP must be followed long-term because of relapses and frequent long-term adverse sequelae.