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Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura

发布于：2013年7月14日    文字：【大】【中】【小】

Blood. 2013 Jul 9. [Epub ahead of print]

Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura.

Deford CC, Reese JA, Schwartz LH, Perdue JJ, Kremer Hovinga JA, L?mmle B, Terrell DR, Vesely SK, George JN.

Source

Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States;

Abstract

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, 1995-2011, who had ADAMTS13 activity <10% with 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 to expected values based on the USreference population. At their initial diagnosis, median age of the 57 survivors was 39 years; 45 (79%) were women; 21 (37%) were black; BMI and prevalence of SLE (7%) were greater (p<0.001) than expected values; prevalence of hypertension (19%, p=0.463) was not different. GFR (all 57 patients at their last follow-up) and ACR (37 of 46 surviving patients in 2011) were not different from expected values (p=0.397, 0.793). In 2011-2012, prevalence of hypertension (40% vs. 23%, p=0.013) and major depression (19% vs. 6%, p=0.005) was greater than expected values. Eleven (19%) patients have died, a proportion greater than expected compared to US andOklahomareference populations (p<0.05). TTP survivors may have greater risk for poor health and premature death.

血栓性血小板减少性紫癜(TTP)急性期的恢复不包括轻微的认知障碍以及复发的危险因素的存在。俄克拉荷马的TTP-HUS登记系统纳入了70个自1995年至2011年连续随访的TTP患者，这些患者首次发作时ADAMTS13活性均低于10%，到2012年，其中57位患者仍然存活。本研究对这些患者的BMI指数、肾小球滤过率(GFR)、尿白蛋白/肌酐比值(ACR)、血压、抑郁症的发生、SLE的发生以及死亡的危险因素的出现均做了统计和比较，参考人群是美国的参照人群。在最初诊断时，这57位存活的患者的中位年龄为39岁，其中45%为女性，21人(37%)为黑人，BMI指数以及SLE的发生率(7%)比预期升高(p<0.001)，高血压的发生率(19%,p=0.463) 与参照人群相比没有显著差异。GFR(57位患者在最后一次随访时的数值)和ACR(46位存活患者中的37位为2011年的数值)与预期数值无统计学差异(p=0.397, 0.793)。2011年至2012年，高血压的发生率(40% vs. 23%, p=0.013)以及抑郁症的发生率(19% vs. 6%, p=0.005)比预期数值升高。11位(19%)患者在2011年死亡，比例明显高于美国及俄克拉荷马的参照人群(p<0.05)。TTP幸存者的非健康状态及早死的危险性可能在增加。