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Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: Comparison of incidence, demographic and clinical features.
发布于：2013年6月13日 文字：【大】【中】【小】
Pediatr Blood Cancer. 2013 Jun 1. doi: 10.1002/pbc.24612. [Epub ahead of print]
Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: Comparison of incidence, demographic and clinical features.
Reese JA, Muthurajah DS, Hovinga JA, Vesely SK, Terrell DR, George JN.
Source
Department of Biostatistics and Epidemiology, College of Public Health; Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma.
Abstract
BACKGROUND:
Thrombotic thrombocytopenic purpura (TTP) associated with severe, acquired ADAMTS13 deficiency is uncommonly reported in children. The incidence, demographic, and clinical features of these children, compared to adults, have not been described.
PROCEDURES:
This study focused on children (<18 years old) and adults with TTP associated with severe, acquired ADAMTS13 deficiency, defined as activity <10%. The incidence rates for TTP in children and adults were calculated from patients enrolled in the Oklahoma TTP-HUS (Hemolytic-Uremic syndrome) Registry, 1996-2012. To describe demographic and clinical features, children with TTP were also identified from a systematic review of published reports and from samples sent to a reference laboratory for analysis of ADAMTS13.
RESULTS:
The standardized annual incidence rate of TTP in children was 0.09?×?106 children per year, 3% of the incidence rate among adults (2.88?×?106 adults per year). Among the 79 children who were identified (one from the Oklahoma Registry, 55 from published reports, 23 from the reference laboratory), TTP appeared to be more common among females, similar to the relative increased frequency of women among adults with TTP, and more common in older children. Clinical data were available on 52 children; the frequency of severe renal failure, relapse, treatment with rituximab, and systemic lupus erythematosus in these children was similar to adults with TTP.
CONCLUSIONS:
TTP associated with severe, acquired ADAMTS13 deficiency is uncommon in children. The demographic and clinical features of these children are similar to the features of adults with TTP.
Pediatr Blood Cancer 2013;9999:1-7. © 2013 Wiley Periodicals, Inc.
ADAMTS13严重获得性缺陷引起的儿童与成人TTP在发病、人口统计学及临床特征上的比较
背景：由于严重ADAMTS13获得性缺陷引起的血栓性血小板性紫癜(TTP)在儿童中的报道并不常见。儿童TTP发病率、人口统计学特点及临床表现与成人患者的比较目前还未有报道。
方法：本研究集中由于严重ADAMTS13获得性缺陷(定义为ADAMTS13活性低于10%)引起的于儿童(<18岁)和成人TTP。儿童与成人患者的发病率自Oklahoma
TTP-HUS登记系统1996-2012年的数据统计而来。为了描述人口统计学及临床表现特点，本研究从已发表的系统综述中以及ADAMTS13检测实验室中寻找儿童TTP患者。
结果：儿童TTP患者的标准化年度发病率为0.09?×?106患儿/年，是成人患者发病率的3%(2.88?×?106 成人/年)。79名儿童患者(1名来自Oklahoma登记系统，55名来自已发表的文献，23名来自标准实验室)中，女性患者多见。这与成人患者性别分布类似。患儿年龄偏大。其中52名患儿的临床资料已知。严重的肾脏损伤、复发、利妥昔单抗治疗以及系统性红斑狼疮的发生率儿童与成人类似。
结论：严重ADAMTS13获得性缺陷引起的TTP在儿童中不常见。TTP患者人口统计学及临床表现特点儿童与成人类似。