The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency
The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency
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DOI：
10.1111/j.1538-7836.2005.01436.x
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发表时间：
2005-07-01
影响因子：
10.4
通讯作者：
George, JN
中科院分区：
文献类型：
--
作者：
Terrell, DR;Williams, LA;George, JN
背景：对血栓性血小板减少性紫癜(TTP)发病率的准确估计对于评估当前治疗所需的资源以及预测开发新治疗的必要性是重要的。以前的估计是间接的，没有报告ADAMTS-13缺乏症患者的数据。目的：探讨临床疑似TTP-溶血性尿毒症综合征(TTP-HUS)、特发性TTP-HUS和ADAMTS-13严重缺乏症(ADAMTS-13)的发生率。方法：从俄克拉荷马州TTP-HUS登记处估算发病率，分析1996年1月1日至2004年6月30日期间因临床疑似TTP-HUS首次发作而接受血浆置换治疗的所有206名连续患者。206例患者中有186例(90%)检测到ADAMTS-13活性。结果：所有临床疑似TTP-HUS患者的年龄-性别-种族标化年发病率为11.29×10(6)(95%CI：9.70-12.88)，特发性TTP-HUS为4.46×10(6)(95%CI：3.43-5.50)，ADAMTS-13严重缺乏(5%活动性)患者为1.74×10(6)(95%CI：1.06-2.41)。在所有这三个类别中，女性和黑人的发病率都更高。在ADAMTS-13严重缺乏的患者中，黑人与非黑人的年龄-性别标化发病率比为9.29(95%CI：4.33~19.93)。结论：已经确定了所有临床怀疑TTP-HUS、特发性TTP-HUS和TTP与ADAMTS-13严重缺陷相关的患者的准确发病率估计。女性和黑人的发病率较高，与他们患其他自身免疫性疾病的风险增加相当。
Background: Accurate estimates of the incidence of thrombotic thrombocytopenic purpura (TTP) are important to assess the resources required for current treatments as well as to anticipate the need to develop new treatments. Previous estimates have been indirect and have not reported data on patients with ADAMTS-13 deficiency. Objective: To determine the incidence of patients with TTP-hemolytic uremic syndrome (HUS) in three categories: all patients with clinically suspected TYP-HUS, patients with idiopathic TTP-HUS, and patients with severe ADAMTS-13 deficiency. Methods: Incidence rates were estimated from the Oklahoma TTP-HUS Registry, analyzing all 206 consecutive patients from January 1, 1996 to June 30, 2004 who were treated with plasma exchange for their initial episode of clinically suspected TTP-HUS. ADAMTS-13 activity was measured in 186 (90%) of the 206 patients. Results: The age-sex-race standardized annual incidence rates were 11.29 x 10(6) (95% Cl: 9.70-12.88) for all patients with clinically suspected TTP-HUS; 4.46 x 10(6) (95% CI: 3.43-5.50) for patients with idiopathic TTP-HUS; and 1.74 x 10(6) (95% CI: 1.06-2.41) for patients with severe ADAMTS-13 deficiency (< 5% activity). In all three categories, the incidence rates were greater for women and for blacks. For patients with severe ADAMTS-13 deficiency, the age-sex standardized incidence rate ratio of blacks to non-blacks was 9.29 (95% Cl: 4.33-19.93). Conclusions: Accurate incidence rate estimates for all patients with clinically suspected TTP-HUS, idiopathic TTP-HUS, and TTP associated with severe ADAMTS-13 deficiency have been determined. The greater incidence among women and blacks is comparable with their increased risk for other autoimmune disorders.