Hemophilia A

on 9.2.09 with 0 comments

  • Quantitative or functional deficiency of factor VIII

  • X-linked recessive; bleeding has been seen in heterozygous women, most likely it’s due to unfavorable mosaicism

  • About 30% of patients have no family history and get the d/o through a new mutation

  • Bleeding characteristics

    • May have life-threatening spontaneous, post-traumatic, or post-surgical bleeding

    • Often see hemarthroses and joints may be crippled if recurrent

    • Minor cuts don’t bleed too much because platelets are normal; petechiae are usually absent

    • The aPTT is prolonged, and bleeding time, PT time, and platelet counts are normal

  • Factor VIII transfusions carried HIV risk until recently

  • Severity relates to amount of functional factor VIII

    • Severe = < 1% of normal factor VIII levels

    • Moderate = 1% to 5% of normal

    • Mild = 6% to 50% of normal

  • About 15% of patients develop Abs to factor VIII

Category: Medical Subject Notes , Pathology Notes



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