Usual Adult Dose for Primary Immunodeficiency Syndrome

Intravenously:
300 to 600 mg/kg, IV, every 3 to 4 weeks

• Initial infusion rate: 1 mg/kg/min
• Maximum infusion rate (if tolerated): 8 mg/kg/min

Subcutaneously:
1.37 x current IV dose in grams / number of weeks between IV doses; administer weekly

• Infuse subcutaneously at 20 mL/hr/site
• Use up to 8 infusion sites simultaneously
• Ensure infusion sites are at least 2 inches (5 cm) apart

Comments:

• Begin subcutaneous dosing one week after the patient's last IV infusion.
• For subcutaneous administration: convert the previous IV dose (in grams) by multiplying by 1.37, then dividing this dose into weekly doses based on the previous IV interval.

Use: Treatment of primary humoral immunodeficiency including, but not limited to, congenital agammaglobulinemia, common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.

Usual Adult Dose for Idiopathic (Immune) Thrombocytopenic Purpura

1 g/kg, IV, on two consecutive days (high dose regimen)
or
0.4 g/kg, IV, on five consecutive days
(total dose 2 g/kg)

• Initial infusion rate: 1 mg/kg/min; increase gradually if tolerated
• Maximum infusion rate: 8 mg/kg/min

Comments:

• If after administration of the first 1 g/kg (high dose regimen), an adequate increase in platelet count is seen at 24 hours, the second dose may be withheld.
• High dose regimen is not recommended if expanded fluid volumes exist or if fluid volume may be a concern.
• Do not administer subcutaneously in idiopathic thrombocytopenic purpura (ITP) patients.

Use: Treatment of patients with idiopathic thrombocytopenic purpura to raise platelet counts to prevent bleeding or allow surgery to be performed.

Usual Adult Dose for Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Loading dose: 2 g/kg, IV, in divided doses, over 2 to 4 consecutive days
Maintenance dose: 1 g/kg, IV, over 1 day; OR 0.5 g/kg, IV, given on 2 consecutive days

• Initial infusion rate: 2 mg/kg/min
• Maintenance infusion rate (if tolerated): 8 mg/kg/min every 3 weeks

Comments:

• Patients at risk of renal dysfunction or thrombosis: administer at the minimum infusion rate practicable.

Uses: Treatment of chronic inflammatory demyelinating polyneuropathy to improve neuromuscular disability and impairment, and for maintenance therapy to prevent relapse.

Usual Adult Dose for Multifocal Motor Neuropathy

Gammagard (R):

IV administration:
Dose range 0.5 to 2.4 grams/kg/month based on clinical response
Initial infusion rate: 0.5 mL/kg/hr (0.8 mg/kg/min)
Maintenance infusion rate: Infusion rate may be advanced if tolerated to 5.4 mL/kg/hr (9 mg/kg/min)

Subcutaneous administration:
Initial Dose is 1.37 x previous intravenous dose divided by # of weeks between intravenous doses.
Maintenance dose is based on clinical response and target IgG trough level.
Initial infusion rate:
40 kg body weight (BW) and greater:
30 mL/site at 20 mL/hr/site.
Under 40 kg BW:
20 mL/site at 15 mL/hr/site
Maintenance infusion rate:
40 kg BW and greater:
30 mL/site at 20 to 30 mL/hr/site.
Under 40 kg BW:
20 mL/site at 15 to 20 mL/hr/site

Usual Pediatric Dose for Primary Immunodeficiency Syndrome

Intravenously:
300 to 600 mg/kg, IV

• Initial infusion rate: 1 mg/kg/min
• Maintenance infusion rate (if tolerated): 8 mg/kg/min every 3 to 4 weeks

Subcutaneously:
1.37 x current IV dose in grams / number of weeks between IV doses; administer weekly

• Initial infusion rate: 10 mL/hr/site
• Maintenance infusion rate; patients under 25 kg: 10 mL/hr/site
• Maintenance infusion rate; patients 25 kg and over: 20 mL/hr/site
• Use up to 6 infusion sites simultaneously
• Ensure infusion sites are at least 2 inches (5 cm) apart

Comments:

• Begin subcutaneous dosing one week after the patient's last IV infusion.
• Convert the previous IV dose (in grams) by multiplying by 1.37, then dividing this dose into weekly doses based on the previous IV interval.

Use: Treatment of primary humoral immunodeficiency including, but not limited to, congenital agammaglobulinemia, common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.

Renal Dose Adjustments

Data not available

• Ensure patients with preexisting renal insufficiency are not volume depleted.
• Discontinue if renal function deteriorates.
• Administer at the minimum infusion rate practicable.

Liver Dose Adjustments

Data not available

Dose Adjustments

• Adjust dose over time to achieve desired clinical response and serum IgG trough level.
• Clinical response is the primary consideration in dose adjustment.
• Measure serum IgG trough as soon as 5 weeks after switching from IV to subcutaneous (SC) to determine if dose adjustment is warranted.
• Target IgG troughs on weekly SC dosing = last IV IgG trough level plus 340 mg/dL.
• Monitor trough IgG every 2 to 3 months to determine if additional dose adjustments are needed.

Measles:

• Patients on routine doses less than 400 mg/kg every 3 to 4 weeks, and at risk of measles exposure (e.g. traveling to a measles endemic area): administer a dose of at least 400 mg/kg just prior to expected measles exposure.
• If a patient has been exposed to measles, administer 400 mg/kg as soon as possible after the exposure.

Precautions

US BOXED WARNINGS:
THROMBOSIS, RENAL DYSFUNCTION and ACUTE RENAL FAILURE

• Thrombosis may occur.
• Thrombosis risk factors include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors.
• For patients at risk of thrombosis, administer at the minimum dose and infusion rate practicable.
• Ensure adequate hydration in patients before administration.
• Monitor for thrombosis signs and symptoms.
• Assess blood viscosity in patients at risk of hyperviscosity.
• Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur in predisposed patients with use of immune globulin intravenous (IGIV).
• Renal dysfunction and acute renal failure occur more commonly in IGIV products containing glucose. This product does not contain sucrose.
• For patients at risk of renal dysfunction or failure, administer at the minimum concentration available and minimum infusion rate practicable.

CONTRAINDICATIONS:

• Anaphylactic or systemic hypersensitivity to human immune globulin
• IgA deficiency with antibodies against IgA and a history of hypersensitivity

Safety and efficacy have not been established in patients younger than 2 years.

• All infections suspected to have been transmitted by this product should be reported to the manufacturer.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Do not give subcutaneously in ITP patients because of the risk of hematoma formation.
• Administer at the minimum infusion rate practicable for patients at risk of renal dysfunction or thrombosis.

Storage requirements:

• Refrigerate; do not freeze

Reconstitution/preparation techniques:

• The manufacturer product information should be consulted.

IV compatibility:

• Incompatible with heparin; do not flush line with heparin.
• The manufacturer product information should be consulted.

Monitoring:

• Periodic renal function and urine output monitoring is particularly important in patients at increased risk of developing acute renal failure.
• Assess renal function, including BUN and serum creatinine at baseline and periodically thereafter.
• Consider baseline assessment of serum viscosity in patients at risk of hyperviscosity, including those with cryoglobulins, markedly high triglycerides, or monoclonal gammopathies, due to increased thrombosis risk.
• Perform confirmatory testing for signs of hemolysis.
• If TRALI is suspected, test for anti-neutrophil antibodies and anti-HLA antibodies in both the product and the patient's serum.