Thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening condition due to hereditary or acquired deficiencies in ADAMTS13, an enzyme responsible for cleaving von Willebrand factor multimers into functional monomers. TTP presents as a microangiopathic hemolytic anemia with a severe thrombocytopenia as well as possible fever, renal dysfunction, and neurologic compromise. In pregnancy, TTP is very difficult to distinguish from the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP), as both may present with hemolytic anemia, thrombocytopenia, and organ failure. An ADAMTS13 assay and antibody screen should be utilized for diagnosis in cases of severe thrombocytopenia (<50,000 platelets) especially when symptoms do not improve after delivery. The treatment for hereditary TTP revolves around weekly plasma transfusions based on platelet counts, while acquired TTP requires plasmapheresis to remove the harmful antibodies from circulation. Recurrence is possible, and future pregnancies should be monitored closely as early proper treatment significantly improves both maternal and fetal outcomes.