The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome
The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome
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DOI：
10.1097/00062752-200309000-00003
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发表时间：
2003-09-01
影响因子：
3.2
通讯作者：
George, JN
中科院分区：
文献类型：
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作者：
George, JN
血栓性血小板减少性紫癜-溶血性尿毒综合征多见于女性，且女性中常与妊娠相关。回顾1964 ~ 2002年血栓性血小板减少性紫癜-溶血性尿毒综合征的病例系列，（1）记录妊娠期间发生血栓性血小板减少性紫癜-溶血性尿毒综合征的报告，（2）检索首次妊娠时首次诊断为先天性或家族性血栓性血小板减少性紫癜-溶血性尿毒综合征的妇女的报告。妊娠期间发生血栓性血小板减少性紫癜-溶血性尿毒综合征的风险最大的时间是足月和产后。这也是血栓事件和其他妊娠相关综合征发生的最大风险时间：先兆子痫，子痫和溶血，肝酶升高，血小板减少（HELLP）综合征。这些其他综合征也可能与血小板减少症、微血管病性溶血性贫血、神经系统症状和肾功能不全有关，使得它们与血栓性血小板减少性紫癜-溶血性尿毒综合征的区别变得困难或不可能。先兆子痫和相关综合征的发生、妊娠晚期和产后发生的高凝状态以及妊娠晚期发生的ADAMTS 13浓度逐渐降低可能联合收割机联合增加血栓性血小板减少性紫癜-溶血性尿毒综合征发生的风险。
Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome occurs more commonly in women and among women is commonly associated with pregnancy. Case series of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome from 1964 to 2002 were reviewed (1) to document the reports of occurrence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy and (2) to search for reports of women with congenital or familial thrombotic thrombocytopenic purpura-hemolytic uremic syndrome who were initially diagnosed during their first pregnancy. The time during pregnancy with greatest risk for development of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is near term and during the postpartum period. This is also the time of greatest risk for thrombotic events and for the occurrence of other pregnancy-related syndromes: preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. These other syndromes may also be associated with thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, and renal insufficiency, making their distinction from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome difficult or impossible. The occurrence of preeclampsia and related syndromes, the hypercoaguable state that occurs in late pregnancy and postpartum, and the progressively decreasing concentration of ADAMTS13 that occurs during late pregnancy may combine to increase the risk for occurrence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.