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Hemophilia with bleeding: Rapid overview of emergency management

Hemophilia with bleeding: Rapid overview of emergency management
Urgent triage
  • Use the patient's bleeding plan if available.
  • Contact the primary hematologist, on-call hematologist, or a hemophilia treatment center (HTC), but if unable to reach them, do not delay treatment while awaiting contact.
  • Contact information for hemophilia treatment centers can be found on the CDC website (https://dbdgateway.cdc.gov/HTCDirSearch.aspx).
Treat first, evaluate second, plan further therapy third
  • For potentially serious or life-threatening bleeding*, give factor before imaging or other evaluations.
    • Initial examination findings may be subtle or absent; treat based on the medical history.
    • If a diagnostic procedure is required (lumbar puncture, arterial blood gas, arthrocentesis), give factor to raise the level to 100% before performing the procedure.
    • If the patient requires transfer to another facility, give factor before (or during) transport.
  • For severe disease, assume the factor level is 0%.
  • Do not waste factor (administer excess rather than discarding).
  • Use an indwelling central catheter to administer factor if present. If not, the most experienced individuals should perform venipunctures and place an intravenous line if needed. Traumatic venipunctures can cause painful hematomas that limit intravenous access.
Give the correct factor product
  • Hemophilia A without an inhibitor – Give factor VIII (factor 8), the patient's own product or recombinant human factor VIII. If not available, use plasma-derived factor VIII or Humate P.
  • Hemophilia B without an inhibitor – Give factor IX (factor 9), the patient's own product or recombinant human factor IX. If not available, use plasma-derived factor IX.
  • Hemophilia A or B with an inhibitor – A bypassing agent such as rFVIIa or FEIBA.
Hemophilia A key points
  • The treatment of choice is the patient's own factor VIII product or recombinant human factor VIII. If neither is available, give plasma-derived factor VIII.
  • For severe bleeding in patients without an inhibitor, give factor VIII at 40 to 50 units/kg as soon as possible to produce a factor VIII level of 80 to 100%. This administration of factor VIII also applies to people using emicizumab prophylaxis. For less-severe joint or muscle bleeding, a target factor VIII level of 40 to 50% may be used, by giving factor VIII at a dose of 20 to 25 units/kg.
  • For severe bleeding in patients with an inhibitor, a bypassing product may be indicated (rFVIIa or FEIBA). Caveats are discussed below.
  • For individuals with mild hemophilia A (baseline factor VIII 5 to 50%) who have a documented response to DDAVP and non-life-threatening (or non-limb-threatening) bleeding, DDAVP may be used.
Hemophilia B key points
  • The treatment of choice is the patient's own factor IX product or recombinant human factor IX. If neither is available, give plasma-derived factor IX.
  • For severe bleeding, give factor IX at 100 to 140 units/kg as soon as possible to produce a factor IX level of 80 to 100%. For less-severe joint or muscle bleeding, a target factor IX level of 40 to 50% may be used, by giving a factor IX at a dose of 50 to 70 units/kg.
  • For patients with an inhibitor, a bypassing product (rFVIIa) may be appropriate for severe bleeding, with caveats discussed below.
Contact the patient's primary hematologist or hemophilia treatment center as soon as possible to assist in management; however, this should not delay prompt administration of the appropriate factor product to treat life-threatening bleeding or bleeding that has the potential to become life-threatening*. Subsequent factor dosing is based on peak and trough levels and calculated using the volume of distribution of the specific product, which is listed separately in UpToDate.

DDAVP: desmopressin; FEIBA: factor eight inhibitor bypassing activity; rFVIIa: recombinant activated factor VII.

* Examples of potentially serious or life-threatening bleeding:

  • Intracerebral bleeding, head trauma, or severe headache.
  • Ocular bleeding.
  • Bleeding into a closed space, target joint, large joint (hip, iliopsoas), or muscle. For bleeds into knees/ankles/elbows, lower dosing may be adequate. If the joint has become a target joint or has chronic bleeding, higher factor levels may be required.
  • Bleeding with the potential for neurovascular compromise.
  • Intraabdominal or gastrointestinal bleeding.
  • Prolonged bleeding that does not respond to home-based therapy.
  • Significant injury such as a motor vehicle accident or fall from a distance of several feet or more.

¶ Bypassing products include:

  • rFVIIa – Recombinant factor VIIa (rFVIIa; NovoSeven RT) is given at a dose of 90 to 120 mcg/kg (rounded to the nearest vial size) every 2 to 3 hours until hemostasis, followed by longer intervals if needed.
  • FEIBA – FEIBA is an activated prothrombin complex concentrate given at a dose of 50 to 100 units/kg every 6 to 12 hours, not to exceed 100 units/kg/dose or 200 units/kg/day, until hemostasis. FEIBA is avoided if possible in individuals with hemophilia A receiving emicizumab (due to risk of thrombosis or thrombotic microangiopathy), and in individuals with hemophilia B and factor IX inhibitors (due to the risk of anaphylaxis).

The patient, family, or caregivers can also provide information on previous responses to bypassing agents. If the person has had a favorable response to 1 of these therapies in the past, it is reasonable to use the same therapy. If the patient has a bleeding plan, follow the plan:

  • rFVIIa is preferred for people with hemophilia A with inhibitors receiving emicizumab, due to a risk of thrombosis or thrombotic microangiopathy with FEIBA.
  • rFVIIa is also preferred for people with hemophilia B with inhibitors, due to the risk of anaphylaxis due to factor IX in FEIBA, especially for individuals with hemophilia B with an inhibitor who have experienced reactions or anaphylaxis upon exposure to factor IX in the past.
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