PMID- 20722701
OWN - NLM
STAT- MEDLINE
DCOM- 20101215
LR  - 20161125
IS  - 1600-0609 (Electronic)
IS  - 0902-4441 (Linking)
VI  - 85
IP  - 5
DP  - 2010 Nov
TI  - Longer survival associated with HLA-A*03, B*14 among 212 hemochromatosis probands 
      with HFE C282Y homozygosity and HLA-A and -B typing and haplotyping.
PG  - 439-47
LID - 10.1111/j.1600-0609.2010.01511.x [doi]
AB  - BACKGROUND: Human leukocyte antigen (HLA) haplotypes may influence iron 
      phenotypes in patients with HFE hemochromatosis and could affect survival. 
      METHODS: We tabulated general characteristics of HLA-A and -B types and 
      haplotypes of HFE C282Y/C282Y probands diagnosed in medical care and analyzed 
      these data to identify HLA survival modifiers. RESULTS: There were 212 probands 
      (130 men, 82 women). Mean follow-up was 12.0 +/- 6.4 yr (0.1-41.2 yr; 34 deaths). 
      HLA-A*03 was more prevalent in men (76.9% vs. 61.0% women; P = 0.0129); 35.4% of 
      men and 29.3% of women had A*03, B*07; and 7.7% of men and 8.5% of women had 
      A*03, B*14. Twenty-three probands had cirrhosis; none had A*03, B*14. Positivity 
      for A*03 or A*03, B*07 was not a significant predictor or modifier of survival. 
      In multiple regression analyses, A*03, B*14 predicted longer survival (P = 
      0.0004). Kaplan-Meier analysis confirmed longer survival in probands with A*03, 
      B*14 (P = 0.0199, log-rank test). After excluding the 23 non-A*03, B*14 probands 
      with cirrhosis, survival of probands with A*03, B*14 was still greater than that 
      of probands without A*03, B*14 (P = 0.0254; log-rank test). Twenty-four years 
      after diagnosis, cumulative survival of probands with and without A*03, B*14 was 
      100% and 58%, respectively. The percentage of deaths due to iron overload was 
      lower in probands with A*03, B*14 (0% vs. 21.9%; P = 0.0392). CONCLUSIONS: In 
      hemochromatosis probands with HFE C282Y/C282Y, survival was longer in those with 
      HLA-A*03, B*14. Earlier age at diagnosis and less severe iron overload in 
      probands with A*03, B*14 could explain this difference.
CI  - (c) 2010 John Wiley & Sons A/S.
FAU - Barton, James C
AU  - Barton JC
AD  - Southern Iron Disorders Center, Birmingham, AL, USA. ironmd@dnamail.com
FAU - Barton, J Clayborn
AU  - Barton JC
FAU - Acton, Ronald T
AU  - Acton RT
LA  - eng
PT  - Journal Article
PL  - England
TA  - Eur J Haematol
JT  - European journal of haematology
JID - 8703985
RN  - 0 (HFE protein, human)
RN  - 0 (HLA-A Antigens)
RN  - 0 (HLA-A*03 antigen)
RN  - 0 (HLA-A3 Antigen)
RN  - 0 (HLA-B Antigens)
RN  - 0 (HLA-B14 Antigen)
RN  - 0 (Hemochromatosis Protein)
RN  - 0 (Histocompatibility Antigens Class I)
RN  - 0 (Membrane Proteins)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - HLA-A Antigens/*analysis
MH  - HLA-A3 Antigen
MH  - HLA-B Antigens/*analysis
MH  - HLA-B14 Antigen
MH  - Haplotypes
MH  - Hemochromatosis/genetics/*immunology/*mortality
MH  - Hemochromatosis Protein
MH  - Histocompatibility Antigens Class I/*genetics
MH  - Histocompatibility Testing
MH  - Homozygote
MH  - Humans
MH  - Iron Overload
MH  - Male
MH  - Membrane Proteins/*genetics
MH  - Middle Aged
MH  - *Mutation, Missense
MH  - Sex Factors
MH  - Survival Rate
EDAT- 2010/08/21 06:00
MHDA- 2010/12/16 06:00
CRDT- 2010/08/21 06:00
PHST- 2010/08/21 06:00 [entrez]
PHST- 2010/08/21 06:00 [pubmed]
PHST- 2010/12/16 06:00 [medline]
AID - EJH1511 [pii]
AID - 10.1111/j.1600-0609.2010.01511.x [doi]
PST - ppublish
SO  - Eur J Haematol. 2010 Nov;85(5):439-47. doi: 10.1111/j.1600-0609.2010.01511.x.