title: Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes.
abstract: Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) represent multiple disorders with diverse etiologies. We compared the gender and race of 335 patients enrolled in the Oklahoma TTP-HUS Registry across 21 years for their first episode of TTP or HUS to appropriate control groups. The relative frequency of women and white race among patients with TTP-HUS-associated with a bloody diarrhea prodrome and the relative frequency of women with quinine-associated TTP-HUS were significantly greater than their control populations. The relative frequency of women and black race among patients with idiopathic TTP and TTP-associated with severe ADAMTS13 deficiency was significantly greater than their control populations. The relative frequency of black race among patients who had systemic lupus erythematosus (SLE) preceding TTP was significantly greater than among a population of patients with SLE, and the relative frequency of black race among patients with other autoimmune disorders preceding TTP was significantly greater than their control population. No significant gender or race disparities were present among patients with hematopoietic stem cell transplantation-associated thrombotic microangiopathy, TTP associated with pregnancy, or TTP associated with drugs other than quinine. The validity of these observations is supported by the enrollment of all consecutive patients across 21 years from a defined geographic region, without selection or referral bias. These observations of different gender and race disparities among the TTP-HUS syndromes suggest the presence of different risk factors and may serve as starting points for novel investigations of pathogenesis.
authors.affiliation: Department of Biostatistics and Epidemiology, College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK 73126-0901, USA. deirdra-terrell@ouhsc.edu
authors.forename: Deirdra R
authors.initials: DR
authors.lastname: Terrell
authors.forename: Sara K
authors.initials: SK
authors.lastname: Vesely
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D001741:Black or African American; D017677:Age Distribution; D016022:Case-Control Studies; D003967:Diarrhea; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008297:Male; D009825:Oklahoma / Q000453:epidemiology / Q000208:ethnology; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000453:epidemiology* / Q000209:etiology; D011803:Quinine / Q000009:adverse effects; D044469:Racial Groups; D012737:Sex Factors
chemical_list: D011803:Quinine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
references.citation: Ann Intern Med. 2001 Dec 18;135(12):1047-51
references.pmid: 11747383
references.citation: Blood. 2010 Feb 25;115(8):1500-11; quiz 1662
references.pmid: 20032506
references.citation: N Engl J Med. 2006 May 4;354(18):1927-35
references.pmid: 16672704
references.citation: Arthritis Rheum. 1995 Sep;38(9):1260-70
references.pmid: 7575721
references.citation: J Clin Apher. 2001;16(4):202-9
references.pmid: 11835417
references.citation: Cancer Epidemiol Biomarkers Prev. 2009 Apr;18(4):1174-82
references.pmid: 19293308
references.citation: Blood. 1991 Jan 15;77(2):306-10
references.pmid: 1898704
references.citation: West J Med. 1991 Sep;155(3):256-9
references.pmid: 1659038
references.citation: Lancet. 2005 Mar 19-25;365(9464):1073-86
references.pmid: 15781103
references.citation: South Med J. 2007 May;100(5):512-4
references.pmid: 17534089
references.citation: Nature. 2001 Oct 4;413(6855):488-94
references.pmid: 11586351
references.citation: Br J Haematol. 2008 May;141(5):696-707
references.pmid: 18422775
references.citation: N Engl J Med. 2009 Oct 22;361(17):1676-87
references.pmid: 19846853
references.citation: Blood. 2010 Jan 14;115(2):379-87
references.pmid: 19861685
references.citation: Blood. 2003 Jul 1;102(1):60-8
references.pmid: 12637323
references.citation: N Engl J Med. 2003 Mar 20;348(12):1170-5
references.pmid: 12646676
references.citation: J Thromb Haemost. 2005 Jul;3(7):1432-6
references.pmid: 15978100
references.citation: Age Ageing. 1994 Sep;23(5):418-20
references.pmid: 7825490
references.citation: Bone Marrow Transplant. 2008 Jun;41(11):917-8
references.pmid: 18545227
references.citation: N Engl J Med. 1998 Nov 26;339(22):1578-84
references.pmid: 9828245