Immunine

Treatment & prophylaxis of bleeding episodes w/ haemophilia B (congenital factor IX deficiency) in adult & childn >6 yr.

IV infusion Individualized dosage. Max rate: 2 mL/min. Early hemarthrosis, muscle bleeding or oral bleeding 20-40 IU/dL. Repeat every 24 hr, at least 1 day until bleeding is resolved or healing is achieved. More extensive haemarthrosis, muscle bleeding or hematoma 30-60 IU/dL. Repeat infusion every 24 hr for 3-4 days or more until pain & acute disability are resolved. Life-threatening haemorrhages 60-100 IU/dL. Repeat infusion every 8-24 hr until threat is resolved. Minor surgery including tooth extraction 30-60 IU/dL every 24 hr at least 1 day until healing is achieved. Major surgery 80-100 IU/dL (pre-& post-op). Repeat infusion every 8-24 hr until adequate wound healing, then continue therapy for at least another 7 days to maintain factor IX activity at 30-60%. Prophylaxis 20-40 IU/kg at 3-4 day intervals.

Hypersensitivity. Heparin allergy or history of heparin induced thrombocytopenia.

Discontinue use if hypersensitivity reactions including early signs eg, hives, generalised urticaria, chest tightness, wheezing, hypotension & anaphylaxis occur. Monitor development of neutralising Ab (inhibitors) in Bethesda Units (B.U). Higher risk of developing thromboembolic complications in low purity prep. Increased risk of anaphylaxis w/ subsequent challenge w/ factor IX in patients w/ inhibitors. Patients w/ signs of fibrinolysis & w/ disseminated intravascular coagulation (DIC); immunodeficiency or increased red cell turnover (eg, haemolytic anaemia). Patient on low Na diet. Possible transmission of infective agents. Perform assay to determine presence of factor IX inhibitor if expected plasma levels are not attained, or if bleeding is not controlled w/ appropriate dose; standard measures to prevent infections including selection of donors, screening of individual donations & plasma pools for specific markers of infection. Evaluate for presence of inhibitor in patients experiencing allergic reactions. Consider appropriate hepatitis A & B vaccination in patients receiving regular/repeated human plasma derived factor IX conc. Initiate appropriate biological testing to patients w/ liver disease, thrombophilia, hypercoagulability states, angina pectoris, coronary disease or acute MI, post-op patients, premature newborns or newborn infants, or patients at risk of thrombotic phenomena or DIC. Risk of serious Parvorirus B19 infection during pregnancy. Lactation. Childn 6-12 yr.

Hypersensitivity or allergic reactions including angioedema, burning & stinging at infusion site, chills, flushing, generalised urticaria, headache, hives, hypotension, lethargy, nausea, restlessness, tachycardia, tightness of chest, tingling, vomiting, wheezing. Nephrotic syndrome following attempted immune tolerance induction in haemophilia B patients w/ factor IX inhibitors & history of allergic reaction. Development of neutralising Ab (inhibitors) to factor IX in patients w/ haemophilia B. Higher risk for thromboembolic episodes eg, MI, DIC, venous thrombosis & pulmonary embolism in patients using low purity prep. Paraesthesia.

Haemostatics

B02BD04 - coagulation factor IX ; Belongs to the class of blood coagulation factors. Used in the treatment of hemorrhage.

D