Which medications in the drug class Antihemophilic Agents are used in the treatment of Hemophilia B?

Updated: Oct 02, 2020
  • Author: Robert A Zaiden, MD; Chief Editor: Srikanth Nagalla, MD, MS, FACP  more...
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Answer

Antihemophilic Agents

These agents are used to control bleeding in hemophilia B or FIX deficiency and to prevent and/or control bleeding in patients with hemophilia A and inhibitors to FVIII.

These are used to control bleeding in mild hemophilia and in some forms of von Willebrand disease.

These agents raise endogenous FVIII levels in mild hemophilia A. Increases as much as 3-fold from the baseline are observed, with peak responses at 30-60 minutes after infusion. [46]

These replace deficient FVIII in patients with hemophilia A. Recombinant products should be used initially and subsequently in all newly diagnosed cases of hemophilia that require factor replacement.

Desmopressin (DDAVP, Stimate)

The main effect of desmopressin is enhancement of water reabsorption in the kidney and smooth muscle constriction. It causes a dose-dependent increase in plasma FVIII and plasminogen activator.

This agent increases the cellular permeability of collecting ducts, resulting in renal reabsorption of water. Tachyphylaxis may occur, even after first dose, but the drug can be effective again after several days.

Anti-inhibitor coagulant complex (Feiba NF, Feiba VH Immuno)

This agent is a freeze-dried sterile human plasma fraction with factor VIII inhibitor bypassing activity. It contains factors II, IX, and X, mainly nonactivated; and factor VII, mainly in the activated form. It may shorten the activated partial thromboplastin time of plasma containing factor VIII inhibitors. Anti-inhibitor coagulant complex is indicated for prevention and control of spontaneous hemorrhage or bleeding during surgical interventions in hemophilia patients who have autoantibodies or alloantibodies to coagulation factors. It is also indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in patients with hemophilia A or B who have developed inhibitors.

Human antihemophilic factor (Hemofil M, Koate-DVI)

FVIII is a protein in normal plasma that is necessary for clot formation and hemostasis. It activates factor X in conjunction with activated FIX; activated factor X converts prothrombin to thrombin, which converts fibrinogen to fibrin, which, with factor XIII, forms a stable clot.

Recombinant human antihemophilic factor (Recombinate, Kogenate, Helixate, Advate)

FVIII is a protein in normal plasma that is necessary for clot formation and hemostasis. It activates factor X in conjunction with activated FIX; activated factor X converts prothrombin to thrombin, which converts fibrinogen to fibrin, which, with factor XIII, forms a stable clot.

Plasma-derived prothrombin complex concentrates/Factor IX complex concentrates (Bebulin, Profilnine SD)

This agent replaces deficient FIX and other factors in the complex.

Plasma-derived coagulation factor IX concentrate (Alpha Nine SD, Mononine, BeneFIX)

This agent replaces deficient FIX and other factors in the complex. AlphaNine SD and Mononine contain only FIX. BeneFIX is a recombinant product.


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