PMID- 19922436
OWN - NLM
STAT- MEDLINE
DCOM- 20100520
LR  - 20230829
IS  - 1538-7836 (Electronic)
IS  - 1538-7836 (Linking)
VI  - 8
IP  - 2
DP  - 2010 Feb
TI  - Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic 
      purpura: evidence for an immunogenetic link.
PG  - 257-62
LID - 10.1111/j.1538-7836.2009.03692.x [doi]
AB  - 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.
FAU - Scully, M
AU  - Scully M
AD  - Department of Haematology, University College of London Hospitals, and 
      Haemostasis Research Unit, University College London, London, UK. 
      m.scully@ucl.ac.uk
FAU - Brown, J
AU  - Brown J
FAU - Patel, R
AU  - Patel R
FAU - McDonald, V
AU  - McDonald V
FAU - Brown, C J
AU  - Brown CJ
FAU - Machin, S
AU  - Machin S
LA  - eng
PT  - Journal Article
DEP - 20091117
PL  - England
TA  - J Thromb Haemost
JT  - Journal of thrombosis and haemostasis : JTH
JID - 101170508
RN  - 0 (HLA Antigens)
RN  - 0 (HLA-DQ Antigens)
RN  - 0 (HLA-DQ beta-Chains)
RN  - 0 (HLA-DQ7 antigen)
RN  - 0 (HLA-DQB1 antigen)
RN  - 0 (HLA-DR Antigens)
RN  - 0 (HLA-DRB1 Chains)
RN  - 0 (HLA-DRB1*11 antigen)
RN  - 0 (HLA-DRB3 Chains)
RN  - 0 (HLA-DRB4 Chains)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Autoimmunity/*genetics
MH  - Case-Control Studies
MH  - Child, Preschool
MH  - Female
MH  - Gene Frequency
MH  - Genetic Predisposition to Disease
MH  - HLA Antigens/*genetics
MH  - HLA-DQ Antigens/genetics
MH  - HLA-DQ beta-Chains
MH  - HLA-DR Antigens/genetics
MH  - HLA-DRB1 Chains
MH  - HLA-DRB3 Chains
MH  - HLA-DRB4 Chains
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Phenotype
MH  - Purpura, Thrombocytopenic, Idiopathic/*genetics/immunology
MH  - Purpura, Thrombotic Thrombocytopenic/*genetics/immunology
MH  - Recurrence
MH  - Registries
MH  - Risk Assessment
MH  - Risk Factors
MH  - Young Adult
EDAT- 2009/11/20 06:00
MHDA- 2010/05/21 06:00
CRDT- 2009/11/20 06:00
PHST- 2009/11/20 06:00 [entrez]
PHST- 2009/11/20 06:00 [pubmed]
PHST- 2010/05/21 06:00 [medline]
AID - S1538-7836(22)11255-9 [pii]
AID - 10.1111/j.1538-7836.2009.03692.x [doi]
PST - ppublish
SO  - J Thromb Haemost. 2010 Feb;8(2):257-62. doi: 10.1111/j.1538-7836.2009.03692.x. 
      Epub 2009 Nov 17.