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Hemophilia

Katie L. Westerfield, DO and Bryan Malave, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) coagulation proteins leading to bleeding tendencies in affected individuals. The majority of cases are due to inh...

DIAGNOSIS

  • History and initial presentation

    • 2/3 of presenting hemophilic patients have a positive family history. All male infants born to known carriers should have factor level testing.

    • Prolonged ble...

TREATMENT

GENERAL MEASURES

  • Avoid aspirin or other NSAIDs.

  • Treat early; acute bleeds should be treated as quickly as possible, preferable within 2 hours (1)[A].

  • For surgical prophylaxis

    • If major surgery ...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

Patient Monitoring

Regular evaluations every 6 to 12 months, including a musculoskeletal evaluation, an inhibitor screen, liver tests, and tests for antibodies to ...

REFERENCES

1
Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al; for Treatment Guidelines Working Group on Behalf of the World Federation of Hemophilia. Guidelines for the management of hemophilia. H...

ADDITIONAL READING

  • Berntorp E, Shapiro AD. Modern haemophilia care. Lancet.  2012;379(9824):1447–1456. [View Abstract on OvidMedline]

  • Fischer K. Prophylaxis for adults with haemophilia: one size ...

CODES

ICD10

  • D66 Hereditary factor VIII deficiency

  • D67 Hereditary factor IX deficiency

  • Z14.01 Asymptomatic hemophilia A carrier

  • Z14.02 Symptomatic hemophilia A carrier

ICD9

  • 286.0 Congenital factor VIII disor...

CLINICAL PEARLS

  • Deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) coagulation proteins leading to bleeding tendencies in affected individuals

  • Deficiencies of factor VIII or factor IX...

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