Further Outpatient Care
See the list below:
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Because immunoglobulin (Ig)G subclass and/or isolated IgA deficiency are observed most commonly in atopic or autoimmune disorders, treating the concomitant diseases is crucial. Adequate treatment for the underlying allergic component may substantially decrease the rate of chronic upper and lower respiratory infections.
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Live viral vaccines are not a risk in partial defects. When the diagnosis of partial defects is established, live viral vaccines can be administered. However, the risk occurs when the exact diagnosis is not known. Until concomitant T-cell deficiency is ruled out, live viral vaccines should be avoided in the patient and in siblings or other children in the house because the attenuated virus is excreted and poses a threat to the severely immunodeficient patient.
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Sorensen et al (1998) showed that a significant percentage of children with specific antibody deficiency develop protective antibody levels to the conjugated pneumococcal vaccine (Prevnar) with subsequent decreased infections. [60]
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In addition, serum IgA subclass levels, IgG subclass levels, or both may increase to normal range in more than 50%. [50] Therefore, regular monitoring of immunoglobulin levels should be done. Conversely, levels may decrease and lose specific immunoglobulin production as some patients progress to common variable immunodeficiency (CVID).
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Do not make a decision to start IVIg treatment lightly. Although its beneficial effect for patients with agammaglobulinemia or hypogammaglobulinemia is not controversial, its use in IgG subclass deficiency or specific antibody deficiency in the presence of normal total Igs is not routine. In 1999, Lawton pointed out that patients, their parents, or the referring physician often insist on a trial of Ig therapy. [61] When this results in a beneficial effect, discontinuation is often difficult because "the child's health and family peace soon become dependent on continuation of the treatment." [61]
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In addition to the high cost of IVIg and the emotional stress of monthly infusions, keep in mind the real possibility of IVIg transmitting unknown infectious agents. Hepatitis C virus transmission has been documented with certain IVIg preparations.
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Families usually agree to a predetermined interval for an empiric trial of IVIg if the clinical problems with recurrent upper and lower respiratory infections appear to be sufficiently severe in patients younger than 4 years. This may occur during the winter, ending in spring. Immunologic testing then can be repeated 3-4 months after the last infusion. Restart treatment only if the antibody response remains abnormal.
Complications
See the list below:
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Prevent chronic upper and lower respiratory disorders by treating underlying allergic disorders (eg, rhinitis, asthma) with aggressive use of antibiotics to manage bacterial infections (eg, sinusitis, bronchitis, pneumonias). Chronic ear infections can cause hearing loss. Watch for mastoiditis.
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No good studies have been conducted to examine the potential benefits of prophylactic antibiotics, given systemically or topically (ie, aerosolized), in patients with B-cell disorder. However, it can be invaluable in selected patients.
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Patients with impaired responses to vaccines may eventually develop CVID, possibly with concomitant T-cell deficiency. Closely follow up such patients and serially assess specific antibody responses (protein and polysaccharide antigens) and IgG subclasses and/or IgA serum levels.
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Some IgA deficiency also occurs in patients with T lymphocyte deficiency such as ataxia-telangiectasia. Live viral vaccines should be avoided in these patients.
Prognosis
See the list below:
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The prognosis of patients with these B-cell disorders depends on the severity of the humoral deficiency and on the extent to which other associated disorders, such as atopic or autoimmune diseases, are involved.
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IVIg therapy offers a good prognosis for patients with the most severe form of antibody deficiency (ie, agammaglobulinemia).
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In many individuals, selective IgA deficiency occurs without respiratory or intestinal symptoms. The role of selective IgG deficiency role in susceptibility to infections is similarly uncertain because many individuals with selective IgG deficiency are asymptomatic.
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Most patients with IgA and IgG subclass deficiency who are younger than 4 years do well with appropriate antibiotic therapy and outgrow their frequent or recurrent infections, especially if they can make specific antibodies. Closely monitor patients who have IgG subclass deficiency that does not improve to assess for relatively complete B-cell deficiency such as CVID. [10]
Patient Education
See the list below:
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Because of the close association with various allergic and autoimmune disorders, parents and patients must be aware of any underlying chronic atopic disorders.
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Possible allergic triggers may require additional intervention with allergy testing and medical treatment.
Tables
Brand(Manufacturer) |
Manufacturing Process |
pH |
Additives (IVIg products containing sucrose are most often associated with renal dysfunction, acute renal failure, and osmotic nephrosis, particularly with preexisting risk factors [eg, history of renal insufficiency, diabetes mellitus, age >65 y, dehydration, sepsis, paraproteinemia, nephrotoxic drugs]) |
Parenteral Form and Final Concentrations |
IgA Content mcg/mL |
Carimune NF (CSL Behring) |
Kistler-Nitschmann fractionation; pH 4, nanofiltration |
6.4-6.8 |
6% solution: 10% sucrose, < 20 mg NaCl/g protein |
Lyophilized powder 3%, 6%, 9%, 12% |
Trace |
Flebogamma (Grifols USA) |
Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization |
5.1-6 |
Sucrose free, contains 5% D-sorbitol |
Liquid 5% |
< 50 |
Gammagard Liquid 10% (Baxter Bioscience) |
Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation |
4.6-5.1 |
0.25M glycine |
Ready-for-use Liquid 10% |
37 |
Gamunex (Talecris Biotherapeutics) |
Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation |
4-4.5 |
Does not contain carbohydrate stabilizers (eg, sucrose, maltose), contains glycine |
Liquid 10% |
46 |
Gammaplex (Bio Products) |
Solvent/detergent treatment targeted to enveloped viruses; virus filtration using Pall Ultipor to remove small viruses including nonenveloped viruses; low pH incubation |
4.8-5.1 |
Contains sorbitol (40 mg/mL); do not administer if fructose intolerant |
Ready-for-use solution 5% |
< 10 |
Iveegam EN (Baxter Bioscience) |
Cohn-Oncley fraction II/III; ultrafiltration; pasteurization |
6.4-7.2 |
5% solution: 5% glucose, 0.3% NaCl |
Lyophilized powder 5% |
< 10 |
Polygam S/D Gammagard S/D (Baxter Bioscience for the American Red Cross) |
Cohn-Oncley cold ethanol fractionation, followed by ultracentrafiltration and ion exchange chromatography; solvent detergent treated |
6.4-7.2 |
5% solution: 0.3% albumin, 2.25% glycine, 2% glucose |
Lyophilized powder 5%, 10% |
< 1.6 (5% solution) |
Octagam (Octapharma USA) 9/24/10: Withdrawn from market because of unexplained reports of thromboembolic events |
Cohn-Oncley fraction II/III; ultrafiltration; low pH incubation; S/D treatment pasteurization |
5.1-6 |
10% maltose |
Liquid 5% |
200 |
Panglobulin (Swiss Red Cross for the American Red Cross) |
Kistler-Nitschmann fractionation; pH 4 incubation, trace pepsin, nanofiltration |
6.6 |
Per gram of IgG: 1.67 g sucrose, < 20 mg NaCl |
Lyophilized powder 3%, 6%, 9%, 12% |
720 |
Privigen Liquid 10% (CSL Behring) |
Cold ethanol fractionation, octanoic acid fractionation, and anion exchange chromatography; pH 4 incubation and depth filtration |
4.6-5 |
L-proline (approximately 250 mmol/L) as stabilizer; trace sodium; does not contain carbohydrate stabilizers (eg, sucrose, maltose) |
Ready-for use liquid 10% |
< 25 |
