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Risk factors for inhibitor formation in haemophilia: a prevalent case-control study.

Publication ,  Journal Article
Ragni, MV; Ojeifo, O; Feng, J; Yan, J; Hill, KA; Sommer, SS; Trucco, MN; Brambilla, DJ; Hemophilia Inhibitor Study,
Published in: Haemophilia
September 2009

Inhibitor formation is a major complication of haemophilia treatment. In a prevalent case-control study, we evaluated blood product exposure, genotype and HLA type on haemophilia A inhibitor formation. Product exposure was extracted from medical records. Genotype was determined on stored DNA samples by detection of virtually all mutations-SSCP (DOVAM-S) and subcycling PCR. HLA typing was performed by PCR amplification and exonuclease-released fluorescence. Cases experienced higher intensity factor, 455 vs. 200 U per exposure, P < 0.005, more frequent central nervous system (CNS) bleeding, seven of 20 (35.0%) vs. one of 57 (1.7%), P = 0.001 and more commonly from inhibitor families, seven of 20 (35.0%) vs. zero of 57 (0%), P < 0.001, and African-American, 12 of 63 (19.0%) vs. six of 117 (5.1%), P = 0.015. Among the latter, CNS bleeding was more commonly the initial bleed, 60% vs. 0%, P < 0.001, and survival was shorter, 14 vs. 38 yr, P = 0.025. Inhibitor formation was uncommon in those with missense mutations, two of 65 (3.1%) vs. 31 of 119 (26.0%), P = 0.008, and unrelated to factor VIII immunogenic epitope, P = 0.388, or HLA type, P > 0.100. Genotype was not associated with race. Time to immune tolerance was shorter for titres <120 vs. > or = 120 BU/mL, six vs. 16 months, P < 0.01, but unaffected by tolerizing dose regimen, P > 0.50. Inhibitor formation is associated with high intensity product exposure, CNS bleeding, African-American race and low frequency of missense mutations. The ideal time to initiate prophylaxis to reduce CNS bleeding and inhibitor formation will require prospective studies.

Duke Scholars

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Published In

Haemophilia

DOI

EISSN

1365-2516

Publication Date

September 2009

Volume

15

Issue

5

Start / End Page

1074 / 1082

Location

England

Related Subject Headings

  • Young Adult
  • Risk Factors
  • Prevalence
  • Male
  • Infant
  • Humans
  • Hemophilia A
  • Genotype
  • Drug Administration Schedule
  • Dose-Response Relationship, Drug
 

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Ragni, M. V., Ojeifo, O., Feng, J., Yan, J., Hill, K. A., Sommer, S. S., … Hemophilia Inhibitor Study, . (2009). Risk factors for inhibitor formation in haemophilia: a prevalent case-control study. Haemophilia, 15(5), 1074–1082. https://doi.org/10.1111/j.1365-2516.2009.02058.x
Ragni, M. V., O. Ojeifo, J. Feng, J. Yan, K. A. Hill, S. S. Sommer, M. N. Trucco, D. J. Brambilla, and D. J. Hemophilia Inhibitor Study. “Risk factors for inhibitor formation in haemophilia: a prevalent case-control study.Haemophilia 15, no. 5 (September 2009): 1074–82. https://doi.org/10.1111/j.1365-2516.2009.02058.x.
Ragni MV, Ojeifo O, Feng J, Yan J, Hill KA, Sommer SS, et al. Risk factors for inhibitor formation in haemophilia: a prevalent case-control study. Haemophilia. 2009 Sep;15(5):1074–82.
Ragni, M. V., et al. “Risk factors for inhibitor formation in haemophilia: a prevalent case-control study.Haemophilia, vol. 15, no. 5, Sept. 2009, pp. 1074–82. Pubmed, doi:10.1111/j.1365-2516.2009.02058.x.
Ragni MV, Ojeifo O, Feng J, Yan J, Hill KA, Sommer SS, Trucco MN, Brambilla DJ, Hemophilia Inhibitor Study. Risk factors for inhibitor formation in haemophilia: a prevalent case-control study. Haemophilia. 2009 Sep;15(5):1074–1082.
Journal cover image

Published In

Haemophilia

DOI

EISSN

1365-2516

Publication Date

September 2009

Volume

15

Issue

5

Start / End Page

1074 / 1082

Location

England

Related Subject Headings

  • Young Adult
  • Risk Factors
  • Prevalence
  • Male
  • Infant
  • Humans
  • Hemophilia A
  • Genotype
  • Drug Administration Schedule
  • Dose-Response Relationship, Drug