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i

Distinguishing lupus anticoagulants from factor VIII inhibitors in haemophilic and non-haemophilic patients

Filetype[PDF-584.20 KB]


  • English
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    Details:

    • Alternative Title:
      Haemophilia
    • Personal Author:
    • Description:
      Introduction:

      Accurate diagnosis of an inhibitor, a neutralizing antibody to infused factor VIII (FVIII), is essential for appropriate management of haemophilia A (HA). Low-titre inhibitors may be difficult to diagnose due to high rates of false-positive inhibitor results in that range. Transient low-titre inhibitors and false-positive inhibitors may be due to the presence of a lupus anticoagulant (LA) or other non-specific antibodies. Fluorescence immunoassay (FLI) to detect antibodies to FVIII is a sensitive method to identify inhibitors in HA. Evaluations of antibody profiles by various groups have demonstrated that haemophilic inhibitors detected by Nijmegen-Bethesda (NBA) and chromogenic Bethesda (CBA) assays correlate with positivity for anti-FVIII immunoglobulin (Ig) G1 and G4.

      Aim:

      This study sought to determine whether FLI could distinguish false-positive FVIII inhibitor results related to LAs from clinically relevant FVIII inhibitors in HA patients.

      Methods:

      Samples from haemophilic and non-haemophilic subjects were tested for LA, specific FVIII inhibitors by NBA and CBA, and anti-FVIII immunoglobulin profiles by FLI.

      Results:

      No samples from LA-positive non-haemophilic subjects were positive by FLI for anti-FVIII IgG4. Conversely, 91% of NBA-positive samples from haemophilia subjects were positive for anti-FVIII IgG4. Two of 11 haemophilia subjects had samples negative for anti-FVIII IgG4 and CBA, which likely represented LA rather than FVIII inhibitor presence.

      Conclusions:

      Assessment of anti-FVIII profiles along with the CBA may be useful to distinguish a clinically relevant low-titre FVIII inhibitor from a transient LA in HA patients.

    • Subjects:
    • Source:
      Haemophilia. 24(5):807-814
    • Pubmed ID:
      30004159
    • Pubmed Central ID:
      PMC6345165
    • Document Type:
      Journal Article
    • Funding:
      CC999999/Intramural CDC HHS/United States
    • Collection(s):
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