The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency

Terrell, D. R.; Williams, L. A.; Vesely, S. K.; Lämmle, B; Hovinga, J. A. K.; George, J. N. (2005). The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. Journal of thrombosis and haemostasis, 3(7), pp. 1432-1436. Blackwell 10.1111/j.1538-7836.2005.01436.x

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Summary. 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 TTP-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 × 106 (95% CI: 9.70–12.88) for all patients with clinically suspected TTP-HUS; 4.46 × 106 (95% CI: 3.43–5.50) for patients with idiopathic TTP-HUS; and 1.74 × 106 (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% CI: 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.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
UniBE Contributor:	Lämmle, Bernhard
Subjects:	600 Technology > 610 Medicine & health
ISSN:	1538-7933
Publisher:	Blackwell
Language:	English
Submitter:	Factscience Import
Date Deposited:	04 Oct 2013 14:51
Last Modified:	05 Dec 2022 14:15
Publisher DOI:	10.1111/j.1538-7836.2005.01436.x
Web of Science ID:	000171451100008
URI:	https://boris.unibe.ch/id/eprint/21289 (FactScience: 5326)