Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.
J Thromb Haemost. 2015 Jun;13 Suppl 1:S216-22. doi: 10.1111/jth.12953.
Patients who have recovered from their acute episode of acquired ADAMTS13-deficient thrombotic thrombocytopenic purpura (TTP) were once thought to have complete recovery except for risk of relapse. Data from previous publications from the Oklahoma TTP-hemolytic uremic syndrome (HUS) Registry are summarized. Patients have decreased cognitive function and increased prevalence of hypertension, systemic lupus erythematosus, major depression, and albuminuria as compared to the expected values from the US population. The proportion of patients that died during the follow-up period was greater than expected based on the US population reference population. Among women who had a pregnancy following recovery from TTP, relapse during pregnancy or postpartum is uncommon, but the occurrence of preeclampsia may be increased. Thirteen of 16 pregnancies in these women resulted in healthy children. Increased morbidity and mortality in TTP patients following recovery suggest that TTP may be more of a chronic disorder than a disorder with acute episodes and complete recovery.
曾认为急性获得性 ADAMTS13 缺乏性血栓性血小板减少性紫癜(TTP)发作后痊愈的患者除了有复发风险外,其余情况与常人无异。本文总结了来自俄克拉荷马 TTP-溶血尿毒综合征(HUS)登记处的以往出版物中的数据。与美国人群的预期值相比,这些患者的认知功能下降,高血压、系统性红斑狼疮、重度抑郁症和白蛋白尿的患病率增加。与美国人群参考值相比,随访期间死亡的患者比例更高。在 TTP 痊愈后怀孕的女性中,妊娠期间或产后复发并不常见,但子痫前期的发生可能会增加。这些女性中有 13 例妊娠最终产下了健康的孩子。TTP 患者痊愈后发病率和死亡率增加表明,TTP 可能更像是一种慢性疾病,而不是一种发作后完全恢复的急性疾病。