Department of Biostatistics and Epidemiology, College of Public Health.
Blood. 2013 Sep 19;122(12):2023-9; quiz 2142. doi: 10.1182/blood-2013-04-496752. Epub 2013 Jul 9.
Recovery from acute episodes of thrombotic thrombocytopenic purpura (TTP) appears complete except for minor cognitive abnormalities and risk for relapse. The Oklahoma TTP-HUS (hemolytic uremic syndrome) Registry enrolled 70 consecutive patients from 1995 to 2011 with ADAMTS13 activity <10% at their initial episode; 57 survived, with follow-up through 2012. The prevalence of body mass index (BMI), glomerular filtration rate (GFR), urine albumin/creatinine ratio (ACR), hypertension, major depression, systemic lupus erythematosus (SLE), and risk of death were compared with expected values based on the US reference population. At initial diagnosis, 57 survivors had a median age of 39 years; 45 (79%) were women; 21 (37%) were black; BMI and prevalence of SLE (7%) were greater (P < .001) than expected; prevalence of hypertension (19%; P = .463) was not different. GFR (P = .397) and ACR (P = .793) were not different from expected values. In 2011-2012, prevalence of hypertension (40% vs 23%; P = .013) and major depression (19% vs 6%; P = .005) was greater than expected values. Eleven patients (19%) have died, a proportion greater than expected compared with US and Oklahoma reference populations (P < .05). TTP survivors may have greater risk for poor health and premature death.
除了轻微的认知异常和复发风险外,急性血栓性血小板减少性紫癜(TTP)发作的患者似乎已完全康复。俄克拉荷马 TTP-HUS(溶血性尿毒症综合征)登记处从 1995 年至 2011 年共登记了 70 例连续患者,这些患者在初次发作时 ADAMTS13 活性<10%;57 例存活,随访至 2012 年。将体重指数(BMI)、肾小球滤过率(GFR)、尿白蛋白/肌酐比值(ACR)、高血压、重度抑郁症、系统性红斑狼疮(SLE)的患病率以及死亡率与基于美国参考人群的预期值进行了比较。在初次诊断时,57 例幸存者的中位年龄为 39 岁;45 例(79%)为女性;21 例(37%)为黑人;BMI 和 SLE(7%)的患病率较高(P<0.001);高血压(19%;P=0.463)的患病率无差异。GFR(P=0.397)和 ACR(P=0.793)与预期值无差异。2011-2012 年,高血压(40%比 23%;P=0.013)和重度抑郁症(19%比 6%;P=0.005)的患病率高于预期值。11 例患者(19%)死亡,与美国和俄克拉荷马州参考人群相比,这一比例高于预期(P<0.05)。TTP 幸存者的健康状况和过早死亡风险可能更大。