González-Mesa Ernesto, Narbona Isidoro, Blasco Marta, Cohen Isaac
Obstetrics and Gynecology Department, Obstetrics and Gynecology Research Group, IBIMA, University Carlos Haya Hospital, Arroyo de Los Angeles Avenue, Málaga, 29011, Spain.
J Med Case Rep. 2013 Apr 29;7:119. doi: 10.1186/1752-1947-7-119.
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.
血栓性血小板减少性紫癜是一种闭塞性血栓性微血管病，并非妊娠所特有，但在妊娠期间发病率会增加。血栓性血小板减少性紫癜的预后很大程度上取决于早期诊断和治疗。由于分娩通常不会使血栓性血小板减少性紫癜病情缓解，所以最初不考虑终止妊娠，尤其是在34周之前，不过在某些情况下，如子痫前期，可能需要终止妊娠。血浆治疗，包括血浆置换，以及类固醇药物用于治疗。如果病情发展不利导致心肺骤停，心肺复苏措施的有效性很大程度上取决于这些措施的早期启动。对于孕妇，不仅需要迅速实施这些措施，还应决定是否通过濒死剖宫产进行胎儿娩出。
我们报告一例30岁初产妇白人女性在妊娠28周时发生血栓性血小板减少性紫癜的病例，其病情迅速恶化导致心肺骤停，经紧急濒死剖宫产术后胎儿存活但母亲死亡。患者入院时无症状，最初未料到会出现如此不利的病情发展。分析检查结果后给予新鲜冷冻血浆和甲泼尼龙治疗，但病情并未改善。考虑进行血浆置换，但很快就发生了心脏骤停。
尽管血栓性血小板减少性紫癜的患病率较低，但孕妇出现贫血、血小板减少和神经功能改变这三联征应指导临床诊断，并应促使检测金属蛋白酶ADAMTS - 13，以排除或确诊血栓性血小板减少性紫癜，并评估最佳治疗方案。如果孕周超过24周的女性发生心肺骤停，若患者在最初四分钟后仍未恢复脉搏，建议进行濒死剖宫产。
