Unfavorable course in pregnancy-associated thrombotic thrombocytopenic purpura necessitating a perimortem Cesarean section: a case report

Abstract

IntroductionThrombotic 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.Case presentationWe 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.ConclusionsDespite 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.

A new biological marker candidate in female reproductive system diseases: Matrix metalloproteinase with thrombospondin motifs (ADAMTS)

“…This disease is more frequently seen in pregnancy. Pregnancyrelated hypercoagulability and decline of ADAMTS13 levels are the reasons (45). Especially in severe ADAMTS13 deficiency, TTP develops during pregnancy (46).…”

Molecular Advances in Preeclampsia and HELLP Syndrome

“…Table 4 presents data from several case reports, collected from our literature research process, indicating the difficulties in setting the definite diagnosis. It should be noted that there are many cases in which the final diagnosis remains vague, or the authors attribute the unsuccessful initial management to a possible coexistence of HELLP/PE and TTP from the beginning [ 43 , 44 , 45 , 46 , 47 , 48 ].…”

“…On the other hand, the personal history of hereditary or acquired TTP can arouse the suspicion of TTP, measurement of ADAMTS13 should also be performed, and in case of levels < 20%, TTP is suspected, and anti- ADAMTS13 autoantibodies should also be measured [ 48 ].…”

“…In case there is no resolution or even worse, there is a deterioration of the clinical manifestations or the laboratory parameters (persistent anemia, neurological symptoms, further thrombocytopenia) in the first days postpartum, TTP is the most probable diagnosis and plasmapheresis should be performed without any further delay. In the literature, there are several case reports in which TTP was diagnosed postpartum because of deteriorating clinical and laboratory parameters after delivery [ 42 , 43 , 44 , 45 , 46 , 47 , 48 ].…”

Perimortem and postmortem caesarean section: a systematic review

“…According to Zepeda et al if the gestational age is less than 20 WG a PMCS is unlikely to improve the situation, if the gestational age is between 20-23 WG it may improve maternal survival, although fetal survival is very unlikely, and if the gestational age is greater than 24 WG the PMCS may improve maternal and neonatal survival [20,21]. United States [23,24,25,26,27] Colombia [2,28,29] Turkey [30,31,32] United Kingdom [33,34] Mexico [18,35] Scotland [36] Lebanon [37] New Zealand [38] Japan [39] Spain [40] Norway [41] China [42] Taiwan/China (2.56%)…”