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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : -2 مورد

Management of bleeding in a patient with hemophilia B (hereditary factor IX deficiency)

Management of bleeding in a patient with hemophilia B (hereditary factor IX deficiency)
Hemophilia treatment center (HTC) or equivalent expertise is essential, especially for patients with inhibitors. However, life-saving treatments should not be delayed while contacting the HTC or awaiting their response. For joint bleeding, standard RICE protocols apply. An antifibrinolytic agent (TXA or EACA) may be helpful for mucosal bleeding (alone if mild, in addition to factor or bypassing product if moderate to severe).

EACA: epsilon aminocaproic acid; FEIBA: factor eight inhibitor bypassing activity; rFVIIa: recombinant activated factor VII; RICE: rest, ice, compression, and elevation; TXA: tranexamic acid; Vd: volume of distribution.

* Most factor IX inhibitors are high responding. Determination is based on historical peak inhibitor titer (not current measurement). If a bypassing agent is used, rFVIIa is preferred; patients with a history of anaphylactoid reactions cannot receive FEIBA because it contains factor IX.

¶ Bleeding severity:
  • Severe – Any bleeding with high risk of mortality, significant morbidity, or significant blood loss. Refer to inset for examples.
  • Moderate – Any bleeding that is not severe but affects a joint or visceral organ. Examples include early hemarthroses in peripheral joints such as knees, elbows, or ankles. Some HTCs treat these as severe bleeding.
  • Mild – Typically cutaneous or dental bleeding amenable to local control.

Δ Calculation depends on inhibitor titer; refer to UpToDate text for details.

◊ Give the patient's own factor product if available, otherwise give recombinant factor IX. Dosing is based on target factor IX activity and Vd, which varies by factor IX product.

If there is sufficient time to do the dosing calculation, use the formula: Dose (units) = % correction × Vd × weight (kg); refer to the UpToDate table on factor IX products for the Vd of specific products.

If there is insufficient time to do the calculation, use:
  • Target 80 to 100% – Give factor IX at 100 to 140 units/kg
  • Target 40 to 50% – Give factor IX at 50 to 70 units/kg

Measure factor IX peak (ideally, 5 to 15 minutes after the first dose) and trough (approximately 8 to 12 hours after the first dose of a standard half-life factor IX product) and plan for next dose at approximately one half-life (adjust based on peak and trough activity). Factor IX half-life is approximately 18 to 24 hours for standard half-life products and longer for extended half-life products; refer to UpToDate table on factor IX products for half-lives of individual factor IX products.

Graphic 144620 Version 2.0