Further Inpatient Care

Inpatient care may be necessary for any serious clinical conditions associated with common variable immunodeficiency (CVID).

Complications

See History for a discussion of complications.

Prognosis

The prognosis for patients with common variable immunodeficiency is reasonably good unless severe autoimmune disease or malignancy develops and if intravenous immunoglobulin (IVIG) replacement therapy is started early before severe lung damage takes place (see Mortality/Morbidity).

The development of granulomatous-lymphocytic lung disease was associated with a worse prognosis and an increased prevalence of lymphoproliferative disease.^[9]

Patient Education

The effort to educate patients and families regarding early signs of infection should be ongoing. The approach in identifying infectious agents and specific antimicrobial therapy needs to be aggressive.

The following may be helpful resources:

The Immune Deficiency Foundation (40 W Chesapeake Avenue, Suite 308, Towson, MD 21204; telephone: 800-296-4433; e-mail: idf@primaryimmune.org)
The Jeffery Modell Foundation and Primary Immune Deficiency Resource Center (Hotline: 1-866-INFO-4-PI)
National Institutes of Health, MedlinePlus, Immune System and Disorders
For teenagers, Nemours Foundation, Immune System
American Academy of Allergy, Asthma, and Immunology, Tips to Remember: Recurrent of unusually severe infections

Questions

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Table 1. Genetic Defects in Common Variable Immunodeficiency

Genetic defect	Chromosomal location	Inheritance	Phenotype	B cells
TNFRSF13B (TACI); approximately 10% of cases	17p11.2	Autosomal dominant (AD)	Common variable immunodeficiency, selective immunoglobulin A deficiency (SIgAD)	< 5% of cases involve absent TACI expression; 95% of cases have normal TACI expression on B cells; low-to-absent IgA levels; autoimmune disease; lymphoproliferative disease; splenomegaly; reduced class-switched memory B cells
TNFRSF13C (BAFF-R); < 1% of cases	22q13.2	Autosomal recessive (AR)	Late-onset, incomplete penetrance	Absent BAFF-R on B-cell surface; reduced class-switched and non–class-switched memory B cells; increased transitional B cells; impaired response to polysaccharide antigen
ICOS; approximately 2% of cases	2q33	AR	Early and late onset	Absent ICOS on activated T cells; reduced class-switched memory B cells; nodular lymphoid hyperplasia; autoimmunity; predisposition to neoplasm
CD19; < 1% of cases	16p11.2	AR	Early and late onset	Low-to-absent CD19 on B cells; reduced class-switched memory B cells; low CD21+ expression on B cells; normal number of CD20+ cells in peripheral blood
CD81; < 1% of cases		AR	Unclear	Impaired Ca⁺⁺ flux after BCR stimulation; autoimmunity; impaired response to protein and polysaccharide antigens; nearly absent CD19 expression
CD20; < 1% of cases		AR	Unclear	Reduced switched memory B cells; impaired somatic hypermutation; impaired response to pneumococcal polysaccharide antigens
CD21; < 1% of cases		AR	Unclear	Reduced class-switched memory B cells; hypogammaglobulinemia; reduced binding of C3d-containing immune complexes and EBV-gp350 to B cells; impaired response to pneumococcal polysaccharide antigens

Table.

Brand (Manufacturer)	Virus Inactivation process	pH; Additives *	Osmolality (mOsm/kg)	Parenteral Form & Final Concentrations	IgA Content µg/ml	Route of administration
Bivigam (Biotest Pharmaceuticals)	Cold ethanol fractionation, solvent/detergent, nanofiltration	4.0-4.6; glycine, polysorbate 80	Unspecified	Liquid 10%	< 200 µg/ml	IV
Flebogamma DIF(Grifols)	Pasteurization, solvent/detergent treatment, nanofiltration, low pH treatment	5.1-6; D-Sorbitol	240-370	Liquid 5%, 10%	< 50 µg/ml in 5% solution, < 100 µg/ml µg/ml in a 10% solution	IV
Gammagard S/D Low IgA (Baxter)	Cold ethanol fractionation, solvent detergent (S/D) treatment	6.4-7.2;Albumin,Glycine,Glucose, PEG, tri-n-butyl phosphate, octoxynol, polysorbate 80	5%: 636; 10%:1250	Lyophilized powder5%, 10%	< 1 µg/ml in a 5% solution	IV
Gammaplex(Bio Products)	Solvent/detergent, nanofiltraion, low pH incubation	4.8-5.1; D- sorbitol, glycine, polysorbate 80	420-500	Liquid 5%	< 10 µg/ml	IV
Octagam (Octapharma)	Cold ethanol fractionation, solvent/detergent, pH4 treatment	5.1-6.0;maltose	310-380	Liquid 5%	< 200 µg/ml	IV
Privigen(CSL Behring)	pH 4 incubation, nanofiltration, depth filtration	4.6-5.0;L-proline	240-440	Liquid 10%	< 25 µg/ml	IV
Gammagard Liquid(Baxter)	Solvent detergent (S/D), nanofiltration, low pH incubation at elevated temp	4.6-5.1; Glycine	240-300	Liquid 10%	37 µg/ml	IV or Subcutaneous
Gamunex-C(Grifolis)	Caprylate precipitation, depth filtration, chromatography, pH 4 incubation	4-4.5; Glycine	258	Liquid 10%	46 µg/ml	IV or Subcutaneous
Gammaked (Kedrion Biopharma)	Caprylate precipitation, depth filtration, chromatography, low pH incubation	4.0-4.5; glycine	258	Liquid 10%	46 µg/ml	IV or Subcutaneous
Cuvitru (Shire)	Fractionation, SD treatment, nanofiltration, low pH treatment	4.6-5.1; glycine	280-292	Liquid 20%	80 µg/ml	Subcutaneous
Hizentra (CSL Behring)	Cold alcohol freactionation, octanoic acid fractionation, and anion exchange chromatography	4.6-5.2; L-proline, polysorbate 80	380	Liquid 20%	< 50 µg/ml	Subcutaneous
HyQvia (Baxter Healthcare)	Solvent/detergent, nanofiltration, lowpH incubation	4.6-5.1; recombinant human hyaluronidase (7.4)	240-300 (recombinatnt human hyaluroidase (290-350)	Liquid 10%	37 µg/ml	Subcutaneous