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Research ArticleResearch and Reports

Past, Present, and Future Options in the Treatment of Hemophilia A

Donna D. Castellone
American Society for Clinical Laboratory Science January 2019, 32 (1) 36-42; DOI: https://doi.org/10.29074/ascls.2018001032
Donna D. Castellone
NewYork Presbyterian Hospital, Columbia Medical Center
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    Table 1.

    Factor levels to manage bleeds


    Severity of Bleed

    Type of Bleed
    Desired FVIII Level
    *Dosage
    Mild hemorrhagesEarly hemarthrosis, epistaxis, gingival bleeding30 IU1–3 doses FVIII
    Major hemorrhagesLate hemarthrosis, muscle bleeds50 IUMany doses, with continuous monitoring of FVIII levels
    Life-threatening bleedMajor trauma or operation, advanced or recurrent hemarthrosis, major gastrointestinal bleeding, head trauma, compromise of limb or compartment syndrome80–100 IUContinuous infusions

    *The formula for patient dosage to determine the number of units of FVIII to correct the activity level is as follows: Dose in FVIII IU = (wt in kg) × (desired FVIII increase (IU)) × (0.5 IU/kg per IU/dL).

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    American Society for Clinical Laboratory Science: 32 (1)
    American Society for Clinical Laboratory Science
    Vol. 32, Issue 1
    1 Jan 2019
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    Past, Present, and Future Options in the Treatment of Hemophilia A
    Donna D. Castellone
    American Society for Clinical Laboratory Science Jan 2019, 32 (1) 36-42; DOI: 10.29074/ascls.2018001032

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    Past, Present, and Future Options in the Treatment of Hemophilia A
    Donna D. Castellone
    American Society for Clinical Laboratory Science Jan 2019, 32 (1) 36-42; DOI: 10.29074/ascls.2018001032
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    • Article
      • LEARNING OBJECTIVES
      • ABSTRACT
      • INTRODUCTION
      • HISTORY OF TREATMENT
      • PRESENT-DAY TREATMENT OPTIONS
      • MANAGEMENT OF HEMOPHILIA
      • TREATMENT IN THE PRESENCE OF INHIBITORS
      • IMMUNE TOLERANCE INDUCTION
      • GENE THERAPY
      • NONFACTOR THERAPY
      • CONCLUSION
      • References
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    Keywords

    • AA - aminocaproic acid
    • AAV - adeno-associated virus
    • AAV5 - adeno-associated virus serotype 5
    • AT - antithrombin
    • BHK - baby hamster kidney
    • BU - Bethesda units
    • CHO - Chinese hamster ovary
    • DDAVP - desmopressin
    • EHL - extended half-life
    • FIX - factor IX
    • FIXa - activated factor IX
    • VII - factor VII
    • FVIII - factor VIII
    • FX - factor X
    • ITI - immune tolerance induction
    • ITRs - inverted terminal repeats
    • IU - international units
    • PEG - polyethylene glycol
    • PUPs - previously untreated patients
    • rFVIIa - recombinant activated factor VII
    • rFvIII - recombinant factor VIII
    • TA - tranexamic acid
    • TFPI - tissue factor pathway inhibitor
    • VWF - von Willebrand factor
    • hemophilia A
    • factor VIII
    • recombinant
    • prophylaxis
    • half-life

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