Differential Diagnoses
-
Severe Combined Immunodeficiency
Tables
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 |
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 |
