Department of Medicine 3, University Medical Center, Mainz, Germany; Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Germany.
Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology and Department of Haematology, St. Olavs Hospital, Trondheim University Hospital, Trondheim, Norway.
Thromb Res. 2015 Feb;135 Suppl 1:S30-3. doi: 10.1016/S0049-3848(15)50437-4. Epub 2015 Feb 9.
The complex relation between thrombotic thrombocytopenic purpura (TTP) and pregnancy is concisely reviewed. Pregnancy is a very strong trigger for acute disease manifestation in patients with hereditary TTP caused by double heterozygous or homozygous mutations of ADAMTS13 (ADisintegrin And Metalloprotease with ThromboSpondin type 1 domains, no. 13). In several affected women disease onset during their first pregnancy leads to the diagnosis of hereditary TTP. Without plasma treatment mother and especially fetus are at high risk of dying. The relapse risk during a next pregnancy is almost 100% but regular plasma transfusion starting in early pregnancy will prevent acute TTP flare-up and may result in successful pregnancy outcome. Pregnancy may also constitute a mild risk factor for the onset of acute acquired TTP caused by autoantibody-mediated severe ADAMTS13 deficiency. Women having survived acute acquired TTP may not be at very high risk of TTP relapse during an ensuing next pregnancy but seem to have an elevated risk of preeclampsia. Monitoring of ADAMTS13 activity and inhibitor titre during pregnancy may help to guide management and to avoid disease recurrence. Finally, TTP needs to be distinguished from the much more frequent hypertensive pregnancy complications, preeclampsia and especially HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet count) syndrome.
简要回顾血栓性血小板减少性紫癜（TTP）与妊娠之间的复杂关系。妊娠是遗传性 TTP（由 ADAMTS13[解整合素金属蛋白酶 13]的双杂合或纯合突变引起）患者急性疾病表现的一个非常强的诱因。在一些受影响的女性中，疾病在第一次妊娠期间发作，导致遗传性 TTP 的诊断。如果不进行血浆治疗，母亲尤其是胎儿有很高的死亡风险。下一次妊娠的复发风险几乎为 100%，但早期妊娠开始的定期血浆输注将预防急性 TTP 发作，并可能导致成功的妊娠结局。妊娠也可能构成由自身抗体介导的严重 ADAMTS13 缺乏引起的急性获得性 TTP 发作的轻度危险因素。患有急性获得性 TTP 的女性在随后的妊娠中再次发生 TTP 的风险似乎并不高，但子痫前期的风险似乎增加。妊娠期间 ADAMTS13 活性和抑制剂滴度的监测可能有助于指导管理并避免疾病复发。最后，TTP 需要与更常见的高血压妊娠并发症（子痫前期，尤其是 HELLP[溶血、肝酶升高、血小板计数低]综合征）区分开来。
