George James N
The University of Oklahoma Health Sciences Center, Hematology-Oncology Section, PO Box 26901, Oklahoma City, OK 73190, USA.
Curr Opin Hematol. 2003 Sep;10(5):339-44. doi: 10.1097/00062752-200309000-00003.
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.
血栓性血小板减少性紫癜-溶血性尿毒症综合征在女性中更为常见，且在女性中通常与妊娠相关。对1964年至2002年血栓性血小板减少性紫癜-溶血性尿毒症综合征的病例系列进行了回顾，（1）记录妊娠期间血栓性血小板减少性紫癜-溶血性尿毒症综合征的发生报告，（2）查找先天性或家族性血栓性血小板减少性紫癜-溶血性尿毒症综合征女性患者在首次妊娠期间首次确诊的报告。妊娠期间发生血栓性血小板减少性紫癜-溶血性尿毒症综合征风险最高的时间是接近足月时和产后期间。这也是发生血栓事件以及其他妊娠相关综合征（先兆子痫、子痫和溶血、肝酶升高、血小板减少 [HELLP] 综合征）风险最高的时间。这些其他综合征也可能与血小板减少、微血管病性溶血性贫血、神经症状和肾功能不全相关，使得它们与血栓性血小板减少性紫癜-溶血性尿毒症综合征难以区分或无法区分。先兆子痫及相关综合征的发生、妊娠晚期和产后出现的高凝状态以及妊娠晚期期间ADAMTS13浓度的逐渐降低可能共同增加血栓性血小板减少性紫癜-溶血性尿毒症综合征的发生风险。
