Unfavorable course in pregnancy-associated thrombotic thrombocytopenic purpura necessitating a perimortem Cesarean...

Thrombotic thrombocytopenic purpura is a type of occlusive thrombotic microangiopathy that is not specific to pregnancy but occurs with an increased frequency during it. Prognosis of thrombotic thrombocytopenic purpura greatly depends on early diagnosis and treatment. As delivery does not generally cause resolution of thrombotic thrombocytopenic purpura, pregnancy termination is not initially considered, especially under 34 weeks, although it may be required under some conditions such as preeclampsia. Plasma therapy, including plasmapheresis, and steroids are used for treatment. In the event of an unfavorable course leading to cardiopulmonary arrest, effectiveness of cardiopulmonary resuscitation measures greatly depends on an early start of such measures. In pregnant patients, not only rapid implementation of these measures is required, but a decision should also be taken about the convenience of fetal delivery through a perimortem Cesarean section.

We report the case of thrombotic thrombocytopenic purpura in a 30-year-old primigravida white woman in week 28 of pregnancy that had a rapidly deteriorating course leading to cardiopulmonary arrest and an emergency perimortem Cesarean section resulting in fetal survival but maternal death. The patient was asymptomatic at admission and such an unfavorable evolution was initially unexpected. Analytical findings were treated with fresh frozen plasma and methylprednisolone but they did not improve. Plasmapheresis was considered but cardiac arrest rapidly ensued.

Despite the low prevalence of thrombotic thrombocytopenic purpura, the finding in a pregnant woman of the triad consisting of anemia, thrombocytopenia, and neurological changes should guide clinical diagnosis, and should prompt measurement of the metalloprotease ADAMTS-13 in order to rule out or confirm diagnosis of thrombotic thrombocytopenic purpura and evaluate the best therapeutic option. If cardiopulmonary arrest occurs in a woman with a gestational age of more than 24 weeks, a perimortem Cesarean section is advised if the patient has not recovered her pulse after the first four minutes.