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.

Contraindications:

IgA-deficient patients with antibodies against IgA. Previous severe reaction to human immune globulin.

Boxed Warning:

Thrombosis.

Warnings/Precautions:

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.

Pharmacologic Class:

Immune globulin.

Interactions:

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.

Adverse Reactions:

Infusion site reactions (eg, erythema, pain, swelling, bruising, nodule, pruritus, induration, scab, edema), cough, diarrhea; hypersensitivity reactions (discontinue if occurs), hemolytic anemia.

Generic Availability:

NO

How Supplied:

Single-use vial (5mL, 10mL, 20mL, 50mL)—1