Document Detail

Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic link.
MedLine Citation:
PMID:  19922436     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, acute, life-threatening disorder, associated with a deficiency in ADAMTS 13. The majority of acute, idiopathic, adult TTP cases are associated with anti-ADAMTS 13 IgG antibodies. However, the factor(s) precipitating an acute TTP episode are not always obvious; indeed, a multifactorial etiology is likely. OBJECTIVES AND METHODS: DNA was used for human leukocyte antigen (HLA) class II typing, using polymerase chain reaction (PCR)-sequence-specific primer and PCR-sequence-specific oligonucleotide probe to methodology to investigate 50 European acquired idiopathic TTP cases. RESULTS: There was an increase in the frequency of HLA-DQB1*0301 (HLA-DQ7) in patients with TTP as compared with controls [58.0% vs. 34.5% (P=0.048)]. The frequencies of HLA-DRB1*11 and HLA-DRB3* were also significantly increased in TTP patients as compared with controls [44.0% vs. 12.0% (P=0.0024) and 84.0% vs. 58.0% (P=0.024)], although it remains uncertain whether susceptibility is influenced by HLA-DQ or HLA-DR molecules or other genes in this haplotype. The frequencies of HLA-DRB1*04 and HLA-DRB4 (HLA-DR53) were significantly decreased in the patient group as compared with controls [10.0% vs. 35.0% and 26.0% vs. 61.5% (P=0.0096 and P=0.0024, respectively)], and may have a protective effect against the development of TTP. CONCLUSION: Analysis identified HLA class II types associated with susceptibility to and a protective effect against the development of acute acquired TTP in European patients. This provides the first description of a genetic factor predicting the risk of developing acquired antibody-mediated TTP.
M Scully; J Brown; R Patel; V McDonald; C J Brown; S Machin
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Publication Detail:
Type:  Journal Article     Date:  2009-11-17
Journal Detail:
Title:  Journal of thrombosis and haemostasis : JTH     Volume:  8     ISSN:  1538-7836     ISO Abbreviation:  J. Thromb. Haemost.     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-02-19     Completed Date:  2010-05-20     Revised Date:  2010-10-15    
Medline Journal Info:
Nlm Unique ID:  101170508     Medline TA:  J Thromb Haemost     Country:  England    
Other Details:
Languages:  eng     Pagination:  257-62     Citation Subset:  IM    
Department of Haematology, University College of London Hospitals, and Haemostasis Research Unit, University College London, London, UK.
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MeSH Terms
Autoimmunity / genetics*
Case-Control Studies
Child, Preschool
Gene Frequency
Genetic Predisposition to Disease
HLA Antigens / genetics*
HLA-DQ Antigens / genetics
HLA-DR Antigens / genetics
Middle Aged
Purpura, Thrombocytopenic, Idiopathic / genetics*,  immunology
Purpura, Thrombotic Thrombocytopenic / genetics*,  immunology
Risk Assessment
Risk Factors
Young Adult
Reg. No./Substance:
0/HLA Antigens; 0/HLA-DQ Antigens; 0/HLA-DQB1 antigen; 0/HLA-DQw7; 0/HLA-DR Antigens; 0/HLA-DRB1*11 antigen; 0/HLA-DRB3; 0/HLA-DRB4; 128338-86-3/HLA-DRB1 antigen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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