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Hemophilia

Discussion

  • classic disease results from deficiency of factor VIII clotting activity;
    • hemophilia A is not caused by an absolute deficiency of factor VIII, but from an abnormal molecule making up part of the VIII clotting factor;
  • hemophilic arthropathy: pathologic changes (coag pathway / coag labs);
  • inheritance pattern:
    • sex-linked recessive;
    • affected male will have normal sons but daughters who are carriers;
    • female carriers may have abnormal coag studies or even mild bleeding diathesis, only affected males demonstrate bleeding problems;

Diff Dx

  • Von Willebrand's disease:
    • autosomal dominant transmission, long bleeding time, decreased factor VIII antigen level, & decrease in ristocetin induced platelet aggregation;
    • infrequently causes musculoskeletal problems, may also exhibit low levels of factor VIII activity;
  • Hemophilia B: (Christmas disease)
    • dx is confirmed by abnormal PTT & low factor IX activity;
    • results from a deficiency of clotting factor IX;
    • although not as common as hemophilia A, factor IX deficiency is similar in that both diseases have sex-linked recessive inheritance, exhibit spontaneous muscle or joint hemorrhages, and commonly produce crippling arthritides;

Clinical Findings

Radiographic Findings in Hemophilia »

Medical Management of Hemophilia »

Management of Specific Joints

Complications

  • HIV infection was common in the past common due to tainted factor VIII;
  • Antibodies to factor VIII;
    • develops in approximately 15% of classical hemophiliacs;
    • these pts have inhibitors to usual replacement therapy & therefore elective surgery is contraindicated

References