Primary immune deficiency:
Indications for XEMBIFY:
Primary humoral immunodeficiency (eg, congenital or X-linked agammaglobulinemia, common variable immunodeficiency, Wiskott-Aldrich syndrome, severe combined immunodeficiencies).
Adults and Children:
<2yrs: not established. Give by SC infusion into abdomen, thigh, upper arm, sides, back and/or lateral hip. May use up to 6 infusion sites (≥2 inches apart) simultaneously; rotate sites with each infusion avoiding bony areas. Before switching to Xembify, obtain serum IgG trough level to guide subsequent dose adjustments. ≥2yrs: Individualize. Switching from IGIV: initiate 1 week after last IGIV; convert monthly dose into equivalent weekly dose. Initial weekly dose = (prior IGIV [in grams]/number of weeks between IGIV doses) × 1.37. Infuse as tolerated up to max 25mL per infusion site at max rate 25mL/hr/site. For frequent dosing (2–7 times per week): divide calculated weekly dose by desired number of times per week. Dose adjustments: measure serum IgG trough levels as early as 5 weeks after initiation and every 2–3 months based on adequate clinical response (see full labeling). Switching from IGSC: use same weekly dose (in grams) as prior IGSC treatment. Thrombosis risk: give at minimum dose and/or infusion rate practicable.
IgA-deficient patients with antibodies against IgA. Previous severe reaction to human immune globulin.
Advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling central venous catheters, hyperviscosity, cardiovascular risk factors: increased risk for thrombosis. Ensure adequate hydration. Monitor for signs/symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity. Pre-existing renal insufficiency, diabetes, >65yrs, hypovolemia, sepsis, paraproteinemia: increased risk of renal dysfunction; monitor and consider lower, more frequent dosing. Correct volume depletion; assess renal function, BUN, serum creatinine, urine output before and during therapy; consider discontinuation if renal function deteriorates. Monitor for aseptic meningitis, hemolysis (esp. with high doses [≥2g/kg], non-O blood group); perform appropriate lab tests. Monitor for pulmonary dysfunction; perform test for anti-neutrophil antibodies if transfusion-related acute lung injury (TRALI) suspected. Contains human plasma; monitor for possible infection transmission (eg, viruses, Creutzfeldt-Jakob disease agent). Elderly. Pregnancy. Nursing mothers.
May interfere with response to live viral vaccines (eg, measles, mumps, rubella, varicella). Concomitant nephrotoxic drugs: increased risk of acute renal failure. May cause false (+) serological test results or (+) direct or indirect Coombs' test.
Infusion site reactions (eg, erythema, pain, swelling, bruising, nodule, pruritus, induration, scab, edema), cough, diarrhea; hypersensitivity reactions (discontinue if occurs), hemolytic anemia.
Single-use vial (5mL, 10mL, 20mL, 50mL)—1