Gamunex-C[免疫球蛋白注射液（人用），10％辛酸酯／色谱纯化]-FDA批准用于皮下注射，治疗原发性免疫缺陷（primary immunodeficiency，PI）。皮下给药途径的获批是对已经获批的用于治疗PI和其他两种适应症（慢性炎性脱髓鞘性多神经病和特发性血小板减少性紫癜）的静脉给药途径的补充。

Immune Globulin IV
( IGIV ) Pronunciation: (im-UM GLOB-u-lin)
Class: Immune globulin
Trade Names:
Carimune NF
- Injection, lyophilized powder for solution immune globulin (human) 3, 6, 12 g

Trade Names:
Flebogamma 5%
- Injection immune globulin (human) 5% (50 mg/mL)

Trade Names:
Gammagard
- Injection immune globulin (human) 10% (100 mg/mL)

Trade Names:
Gammagard S/D
- Injection immune globulin (human) 5% (50 mg/mL)

Trade Names:
Gamunex
- Injection immune globulin (human) 10% (100 mg/mL)

Trade Names:
Iveegam EN
- Injection, freeze-dried powder for solution immune globulin (human) 5 g

Trade Names:
Octagam
- Injection immune globulin (human) 5% (50 mg/mL)

Trade Names:
Privigen
- Injection immune globulin (human) 10% (100 mg/mL)

Gammagard S/D (Canada)
Sandoglobulin NF Liquid (Canada)

HOW SUPPLIED:

10mL - Injectable Vial

25mL - Injectable Vial

50mL - Injectable Vial

100mL - Injectable Vial

200mL - Injectable Vial

Pharmacology

Replaces normal human immunoglobulin (Ig) G antibodies. Promotes opsonization, fixes complement, and neutralizes bacteria, viruses, fungi, and parasites, and their toxins.

Indications and Usage
Treatment of primary immunodeficiency states in patients unable to produce sufficient amounts of IgG antibodies.

Carimune NF
Maintenance treatment of patients with primary immunodeficiencies (eg, common variable immunodeficiency).

Carimune NF, Gamunex
Idiopathic thrombocytopenic purpura (ITP). Consider use in situations that require a rapid, temporary rise in platelet count (eg, prior to surgery).

Flebogamma, Gamunex
Primary (inherited) humoral immunodeficiency disorders (eg, variable immunodeficiency, X-linked agammaglobulinemia, severe combined immunodeficiency, Wiskott-Aldrich Syndrome).

Gammagard
Primary immunodeficiency diseases (congenital X-linked gammaglobulinemia, common variable immunodeficiency, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies).

Gammagard S/D
Primary immunodeficiency diseases; B-cell chronic lymphocytic leukemia (CLL); ITP; Kawasaki syndrome.

Gamunex
Treatment of chronic inflammatory demyelinating polyneuropathy (CIDP).

Ivvegam EN
Primary immunodeficiency diseases (eg, common variable immunodeficiency, X-linked agammaglobulinemia, hyper IgM syndromes, and Wiskott-Aldrich syndrome); Kawasaki syndrome.

Octagam
Treatment of primary immune deficient diseases (eg, congenital agammaglobulinemia and hypogammaglobulinemia, Wiskott-Aldrich syndrome).

Privigen
Primary immunodeficiency diseases; chronic immune thrombocytopenia purpura.

Unlabeled Uses
Posttransfusion purpura, Guillain-Barre syndrome, and chronic inflammatory demyelinating polyneuropathy (as an alternative to plasma exchange). Prevention or treatment of autoimmune diseases (eg, rhesus hemolytic disease, rheumatoid arthritis), IgG4 subclass deficiencies, intractable epilepsy, cystic fibrosis, trauma, thermal injury (eg, severe burns), cytomegalovirus infection, neuromuscular disorders; prophylaxis of infections associated with bone marrow transplantation; GI protection; multiple sclerosis; myasthenia gravis.

Contraindications
Immediate hypersensitivity to human antibody product; selective IgA deficiency and circulating anti-IgA antibodies; possible aseptic meningitis syndrome; hypersensitivity or intolerance to any component of the product. History of severe or anaphylactic reaction to blood or blood-derived products.

Dosage and Administration
B-Cell CLL
Adults and Children Gammagard S/D
IV 400 mg/kg every 3 to 4 wk.

CIDP
Adults Gamunex
IV

Loading dose
2 g/kg (20 mL/kg) in divided doses over 2 to 4 consecutive days.

Maintenance dose
1 g/kg (10 mL/kg) over 1 day, or divided into 2 doses of 0.5 g/kg (5 mL/kg) given on 2 consecutive days, every 3 wk.

Immunodeficiency Syndrome
Adults and Children Carimune NF
IV 0.2 g/kg once a month. (If clinical response is inadequate, increase dose to 0.3 g/kg or repeat infusion more frequently than once a month.)

ITP
Adults and Children Carimune NF
IV For induction therapy, 0.4 g/kg on 2 to 5 consecutive days.

Carimune NF
IV

Maintenance- Chronic ITP
If after induction therapy the platelet count falls below 30,000/mcL and/or the patient manifests bleeding, 0.4 g/kg may be given as a single infusion. If an adequate response does not result, the dose can be increased to 0.8 to 1 g/kg given as a single infusion.

Gammagard S/D
IV 1 g/kg. Additional doses of up to 3 separate doses may be given on alternate days if needed, based on clinical response and platelet count.

Gamunex
IV 2 doses of 1 g/kg given on 2 consecutive days or 5 doses of 0.4 mg/kg given on 5 consecutive days. If, after the first of 2 daily 1 g/kg doses, an adequate increase in platelet count is observed at 24 h, withhold the second 1 g/kg dose.

Adults and Children older than 15 yr of age Privigen
IV Two 1 g/kg doses on 2 consecutive days.

Children Carimune NF
IV

Acute ITP Childhood
If an initial platelet count response to the first 2 doses is adequate (30,000 to 50,000/mcL), therapy may be discontinued after the second day of the 5-day course.

Kawasaki Syndrome
Adults and Children Gammagard S/D
IV 1 g/kg as a single dose or 400 mg/kg for 4 consecutive days beginning within 7 days of the onset of fever, administered with aspirin 80 to 100 mg/kg/day in 4 divided doses.

Iveegam EN
400 mg/kg daily for 4 consecutive days or as a single 2 g/kg dose given over a 10-h period initiated within 10 days of disease onset. Administer with aspirin 80 to 100 mg/kg/day.

Primary Immunodeficiency Diseases
Adults and Children Flebogamma , Gammagard , Gammagard S/D , Gamunex , Octagam
IV 300 to 600 mg/kg administered every 3 to 4 wk. Adjust dose over time to achieve desired trough levels and clinical response.

Iveegam EN
200 mg/kg per month. Dose may be increased up to 4-fold or intervals between infusions shortened if desired results are not obtained.

Adults and Children 3 yr of age and older Privigen
IV 200 to 800 mg/kg at a dosing interval of 3 or 4 wk for 12 mo.

General Advice
For administration by IV infusion only. Not for intradermal, subcutaneous, IM, IV bolus, or intra-arterial administration.
Reconstitute powder for injection immediately before use following manufacturer's guidelines for reconstitution. Do not shake during reconstitution process to avoid foaming.
Contents of individual vials may be pooled under aseptic conditions into sterile infusion containers.
Carefully follow recommended infusion rates to reduce risk of infusion-related adverse reactions (eg, changes in BP and pulse, flushing). Generally, infusions are started at low rates and then increased as tolerated. If adverse reactions occur, the infusion rate may be reduced, or the infusion interrupted until symptoms subside, and then resumed at the rate that is comfortable for the patient.
Ensure that immune globulin IV solutions that have been refrigerated are allowed to come to room temperature before infusing.
Infuse immune globulin IV through separate IV line. Do not add any other medications or IV fluids to immune globulin IV infusion container. If other medications or IV fluids will be sequentially administered, flush IV line with dextrose 5% in water before and after infusion of immune globulin IV. Gamunex may be diluted with dextrose 5% in water if dilution is required.
In-line filtration is acceptable but not required. Pore sizes of 15 microns or more will be less likely to slow infusion.
Do not mix immune globulin IV products of different formulations or from different manufacturers.
Do not administer if solution is discolored, cloudy, or turbid, or if particulate matter is noted.
Gamunex is incompatible with saline.
Do not mix immune globulin IV with other medications.
Storage/Stability
Protect immune globulin IV solutions from freezing. Do not use any immune globulin IV solution that has been frozen.
Store Gamunex for up to 36 mo at 34° to 46°F. Gamunex also may be stored at temperatures not exceeding 77°F for up to 6 mo any time during the 36-mo shelf life, after which the product must be immediately discarded. Do not freeze.

Drug Interactions
Live vaccines
To avoid inactivating vaccines containing live viruses or bacteria, give live vaccines 2 to 4 wk before or 3 to 11 mo after immune globulin IV, depending on dose.

Laboratory Test Interactions
Blood type
Blood-group antibodies may be transferred to immune globulin IV recipients, causing confusion regarding recipient's blood type.

Octagam
Contains maltose, which may be misinterpreted as glucose by certain types of blood glucose testing systems (eg, systems based on glucose-dye-oxidoreductase methods).

Adverse Reactions
Cardiovascular
Cardiac arrest, hypertension, hypotension, thromboembolism, vascular collapse (postmarketing).

CNS
Aseptic meningitis, asthenia, coma, dizziness, fatigue, headache, loss of consciousness, pyrexia/hyperthermia, seizures, tremor (postmarketing).

Dermatologic
Bullous dermatitis, diaphoresis, ecchymosis, epidermolysis, erythema multiforme, flushing, petechiae, pruritus, purpura, rash, Stevens-Johnson syndrome, urticaria (postmarketing).

EENT
Ear pain, epistaxis, nasal congestion, nasopharyngitis, rhinorrhea, sore throat.

GI
Abdominal pain, diarrhea, increased conjugated bilirubin, nausea, stomach discomfort, vomiting.

Genitourinary
Acute renal failure, acute tubular necrosis, osmotic nephrosis, proximal tubular nephropathy.

Hematologic-Lymphatic
Decreased hematocrit, hemolysis, hemolytic anemia, hemorrhage, leukopenia, pancytopenia, positive direct antiglobulin (Coombs) test, thrombocytopenia (postmarketing).

Hepatic
Hepatic dysfunction (postmarketing).

Lab Tests
Increased BUN and creatinine.

Local
Injection-site reaction.

Musculoskeletal
Arthralgia, back pain, leg cramps, limb pain, neck pain, rigors (postmarketing).

Respiratory
Acute respiratory distress syndrome, apnea, asthma, bronchitis, bronchospasm, cyanosis, dyspnea, hypoxemia, increased cough, pharyngitis, pulmonary edema, rhinitis, sinusitis, transfusion-related acute lung injury, upper respiratory tract infection, wheezing (postmarketing).

Miscellaneous
Accidental injury, chills, fever, pain, pain in extremities, tightness in chest.

Precautions

Warnings
Immune globulin products have been associated with renal function impairment, acute renal failure, osmotic nephrosis, and death. Products containing sucrose as a stabilizer account for a disproportionate share of the total number of cases of renal function impairment and acute renal failure. For patients judged to be at increased risk of developing renal function impairment, it may be prudent to reduce amount of product infused per unit time. Do not exceed recommended doses, and ensure that the concentration and infusion rate are at the minimum level practicable.
 
Monitor
Monitor for signs and symptoms of hemolysis and pulmonary adverse reactions. Consider a baseline assessment of blood viscosity in patients at risk for hyperviscosity. Periodically monitor renal function and urinary output.

Pregnancy
Category C .

Lactation
Undetermined.

Children
Flebogamma
Safety and efficacy not established.

Gammagard
Safety and efficacy not established in neonates or infants.

Gamunex treatment of CIDP
Safety and efficacy not established.

Privigen Primary immunodeficiency
Safety and efficacy not established in children younger than 3 yr of age.

Chronic ITP
Safety and efficacy not established in children younger than 15 yr of age.

Elderly
Use with caution in patients older than 65 yr of age and judged to be at increased risk of developing renal function impairment.

Hypersensitivity
Hypersensitivity, including anaphylaxis, may occur.

Renal Function
Use with caution in patients with preexisting renal insufficiency and in patients judged to be at risk of developing renal function impairment. Has been associated with renal function impairment, acute renal failure, osmotic nephrosis, and death. Ensure that immune globulin IV is administered at the minimum concentration available and at the minimum rate of infusion practical in patients predisposed to acute renal failure. Ensure that renal function (BUN, serum creatinine) is evaluated before starting therapy and periodically thereafter in patients determined to be at risk for acute renal failure. Monitor urine output during infusion of immune globulin IV. Be prepared to discontinue therapy.

Aseptic meningitis
Aseptic meningitis syndrome (including drowsiness, fever, nausea, painful eye movements, photophobia, rigidity at nape of neck, severe headache, and vomiting) may occur.

Hemolysis
Positive direct antiglobulin reaction and hemolysis may occur. Monitor patient for signs and symptoms of hemolysis (eg, jaundice, splenomegaly, unexplained fatigue).

Hyperproteinemia
Hyperproteinemia, increased serum viscosity, and hyponatremia may occur.

Hypogammaglobulinemia
Patients with agammaglobulinaemia or extreme hypogammaglobulinemia who have never received immunoglobulin substitution treatment or whose time from last treatment is more than 1 mL/min may be at risk of developing inflammatory reactions with an infusion rate of more than 1 mL/min.

Infection transmission
Immune globulin IV is made from human plasma and may contain infectious agents, such as viruses, that can cause disease.

Thrombotic events
May occur.

Transfusion-related acute lung injury (TRALI)
Noncardiogenic pulmonary edema (TRALI) may occur. Monitor patient for adverse pulmonary reactions. Ensure that tests for antineutrophil antibodies in both the immune globulin IV and patient are performed and evaluated.

美国FDA批准Gamunex治疗神经疾病CIDP

美国FDA批准塔Talecris Biotherapeutics公司的10％ 辛酸盐/色谱分离纯化人免疫球蛋白静脉注射液[（Immune Globulin Intravenous（Human），Gamunex]用）用于治疗慢性炎性脱髓鞘性多发性神经根神经病（CIDP）。CIDP是退行性神经疾病，可造成肌肉无力和乏力，导致运动技能严重损伤。

本品获准上市成为迄今唯一治疗CIDP的药品、美国迄今首个和唯一治疗神经疾病的免疫球蛋白静脉注射液。Talecris Biotherapeutics公司享有本品7年市场专卖权。

本品是含有数以千计捐血者血浆纯化的抗体免疫球蛋白静脉注射液，适用于替代治疗原发性激素免疫缺乏疾病（PI）、免疫调节治疗特发性血小板减少性紫癜（ITP）和CIDP。