TTP 是一种罕见疾病。一项研究中,根据年龄和性别标准化的 TTP 加上溶血性尿毒综合征的发病率在美国是每年每百万人口 6.5 例,在英国是每年每百万人口 2.2 例,在加拿大萨斯喀彻温省是每年每百万人口 3.2 例。[7]Miller D, Kaye JA, Shea K, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004;15:208-215.http://www.ncbi.nlm.nih.gov/pubmed/15127914?tool=bestpractice.com 另一项研究估计在美国 TTP 的发病率为每年每百万人口 4-11 例。[8]Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005;3:1432-1436.http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2005.01436.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15978100?tool=bestpractice.com 有证据表明,TTP 的发病率正在逐渐升高。[9]Torok TJ, Holman RC, Chorba TL. Increasing mortality from thrombotic thrombocytopenic purpura in the United States - analysis of national mortality data, 1968-1991. Am J Hematol. 1995;50:84-90.http://www.ncbi.nlm.nih.gov/pubmed/7573005?tool=bestpractice.com
TTP 和溶血性尿毒综合征在女性和黑人中的发生率更高。[8]Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005;3:1432-1436.http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2005.01436.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15978100?tool=bestpractice.com[7]Miller D, Kaye JA, Shea K, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004;15:208-215.http://www.ncbi.nlm.nih.gov/pubmed/15127914?tool=bestpractice.com 在美国,65% 至 75% 的 TTP 患者是女性,且多达 44% 的患者是黑人。
病因为重度 von Willebrand 因子裂解酶 (ADAMTS-13) 缺乏症的大多数 TTP 患者年龄在 20 岁至 59 岁之间。[8]Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005;3:1432-1436.http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2005.01436.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15978100?tool=bestpractice.com 另外一个报告的平均年龄是 39 岁(范围:19 岁至 71 岁)。[10]Sadler JE, Moake JL, Miyata T, et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2004;407-423.http://asheducationbook.hematologylibrary.org/content/2004/1/407.fullhttp://www.ncbi.nlm.nih.gov/pubmed/15561695?tool=bestpractice.com