Agammaglobulinemia Workup

  • Author: Terry W Chin, MD, PhD; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: Sep 28, 2010
 

Laboratory Studies

In patients with agammaglobulinemia, or hypogammaglobulinemia, all circulating immunoglobulin (Ig) levels (IgG, IgA, IgM, IgE) are low. The physician must compare the patient's specific levels with age-appropriate controls.

Serum IgG levels lower than 100 mg/dL should arouse concern. In some patients with X-linked agammaglobulinemia (XLA), IgG levels may be as high as 200-300 mg/dL. This does not necessarily exclude a diagnosis of XLA.

Patients are also unable to make specific antibody responses. They usually revealed decreased antibody levels against common childhood vaccine antigens such as diphtheria, pertussis, varicella, hepatitis B, and H influenzae.

In young infants (< 6 mo), because the serum IgG level is unreliable secondary to the presence of a maternal antibody, the physician cannot rely on Ig level determinations. Patients' families also have anxiety about a diagnosis of possible immunodeficiency. Determining diphtheria and tetanus antibody titers prior to vaccine administration and after administration in 3-4 week intervals to assess responses. If specific diphtheria and tetanus levels rise, this indicates that the infant is able to produce antigen-specific antibody, rendering agammaglobulinemia (or any other B-cell deficiency) unlikely.

Functional IgM production can be measured by checking isohemagglutinin titers.

Note that pre–B cells can produce IgM in detectable quantities, including IgM autoantibodies particularly directed against hematopoietic cells (typical antirhesus [anti-Rh] in autoimmune hemolytic anemia, antineutrophil antibodies).

Because B-cell maturation is arrested, patients lack mature B lymphocytes in their peripheral blood or tissue. Performing flow cytometry to analyze B- and T-cell marker expression is necessary.

This can be assessed by staining for B-lymphocyte–specific surface cell markers by flow cytometry. Most laboratories should be able to perform this test because similar technology examines the T-lymphocyte markers of CD4 and CD8 used in assessing HIV infection. However, laboratory personnel must be informed that B-lymphocyte–specific monoclonal antibodies (CD19 and/or CD20) should be used for analysis.

Reduced numbers of peripheral blood B lymphocytes suggest the diagnosis, no matter what the age of the patient.

Mutational analysis must be performed to confirm the specific type of agammaglobulinemia.

In addition, plasma cells and B lymphocytes in lymphoid follicles and in germinal centers of lymph nodes may be lacking. Because intestinal biopsy may be obtained to evaluate patients with chronic diarrhea, examination for hypoplastic Peyer patches in the lamina propria of intestinal mucosa may be helpful in diagnosing agammaglobulinemia.

Patients with growth hormone deficiency have a deficient growth hormone response to insulin, arginine, or levodopa (L-dopa). Plasma somatomedin levels are also reduced.

Next

Imaging Studies

No radiological findings are specific for agammaglobulinemia, although it is suggested by an absence of adenoidal tissue (eg, adenoidal tissue in lateral head films to evaluate chronic sinusitis). Chest radiography findings of unexplained bronchiectasis should also lead to an evaluation of the patient's immune status.

High resolution CT scanning of the chest is helpful to delineate the extent of lung damage. One study found bronchiectasis in 58% patients with agammaglobulinemia.[37] However, annual examinations may not be needed because another study suggests no significant progression over a 3-year period.[38]

Sinus CT examinations may be required as clinically needed.[37]

Some physicians advocate using MRI of the brain in patients with agammaglobulinemia or hypogammaglobulinemia who develop unexplained neurological symptoms and signs of meningeal inflammation, despite extensive investigation of cerebrospinal fluid (CSF), including polymerase chain reaction (PCR) analyses.

Delayed bone age is evident in patients with growth hormone deficiency.

Previous
Next

Other Tests

Pulmonary function tests are evaluated at diagnosis because the literature suggests that decreased parameters at diagnosis of hypogammaglobulinemia correlate with chronic and progressive pulmonary disease.

Previous
Next

Histologic Findings

Findings of hypoplastic or absent tonsils, adenoids, and lymph nodes in tissue usually rich in B lymphocytes suggest the diagnosis.

Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Terry W Chin, MD, PhD  Associate Director, Pediatric Allergy/Immunology/Pulmonology, Miller Children's Hospital, Long Beach Memorial Medical Center; Associate Professor, Department of Pediatrics, University of California, Irvine, School of Medicine

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Ann O'Neill Shigeoka, MD †  Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine

Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

John Wilson Georgitis, MD  Consulting Staff, Lafayette Allergy Services

John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Conley ME, Famer DM, Dobbs AK, et al. A minimally hypomorphic mutation in Btk resulting in reduced B cell numbers but no clinical disease. Clin Exp Immunol. 2008;152:39-44.

  2. Ferrari S, Lougaris V, Caraffi S, et al. Mutations of the Igbeta gene cause agammaglobulinemia in man. J Exp Med. Sep 3 2007;204(9):2047-51. [Medline].

  3. Litzman J, Brysova V, Gaillyova R, et al. Agammaglobulinaemia in a girl with a mosaic of ring 18 chromosome. J Paediatr Child Health. Feb 1998;34(1):92-4. [Medline].

  4. Ohga S, Nakao F, Narazaki O, et al. Hypogammaglobulinaemia in a patient with ring chromosome 21. Arch Dis Child. Sep 1997;77(3):252-4. [Medline].

  5. Revy P, Busslinger M, Tashiro K, et al. A syndrome involving intrauterine growth retardation, microcephaly, cerebellar hypoplasia, B lymphocyte deficiency, and progressive pancytopenia. Pediatrics. Mar 2000;105(3):E39. [Medline].

  6. Roifman CM. Antibody deficiency, growth retardation, spondyloepiphyseal dysplasia and retinal dystrophy: a novel syndrome. Clin Genet. Feb 1999;55(2):103-9. [Medline].

  7. Stewart DM, Tian LL, Notaranelo LD, Nelson DL. X-linked hypogammaglobulinemia and isolated growth hormone deficiency: an update. Immunol Rev. 2008;40:262-70. [Medline].

  8. al-Attas RA, Rahi AH. Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia. J Clin Immunol. Sep 1998;18(5):368-71. [Medline].

  9. Mila J, Matamoros N, Pons de Ves J, et al. [The Spanish Registry of Primary Immunodeficiencies. REDIP-1998]. Sangre (Barc). Apr 1999;44(2):163-7. [Medline].

  10. Leal RC, Bertelli EC, Soler ZA. Recurrent pneumonia caused by genetic immunodeficiency: a prophylactic and rehabililtative approach. Braz J Infect Dis. 2007;11:307-10. [Medline].

  11. Eley BS, Hughes J, Cooper M, Pienaar S, Beatty DW. Primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital. S Afr Med J. Dec 1997;87(12):1684-8. [Medline].

  12. Baumgart KW, Britton WJ, Kemp A, et al. The spectrum of primary immunodeficiency disorders in Australia. J Allergy Clin Immunol. Sep 1997;100(3):415-23. [Medline].

  13. Lam DS, Lee TL, Chan KW, et al. Primary immunodeficiency in Hong Kong and the use of genetic analysis for diagnosis. Hong Kong Med J. Apr 2005;11(2):90-6. [Medline].

  14. Freeman AF, Holland SM. Persistent bacterial infections and primary immune disorders. Curr Opin Microbiol. 2007;10:70-5. [Medline].

  15. Mamishi S, Shahmahmoudi S, Tabatabaie H, et al. Novel BTK mutation presenting with vaccine-associated paralytic poliomyelitis. Eur J Pediatr. Nov 2008;167(11):1335-8. [Medline]. [Full Text].

  16. Hidalgo S, Garcia Erro M, Cisierna D, Freire MC. Paralytic poliomyelitis caused by a vaccine-derived polio virus in an antibody-deficient Argentinean child. Pediatr Infect Dis J. 2003;22:570-2. [Medline].

  17. Rudge P, Webster AD, Revesz T, et al. Encephalomyelitis in primary hypogammaglobulinaemia. Brain. Feb 1996;119 ( Pt 1):1-15. [Medline].

  18. Katamura K, Hattori H, Kunishima T, et al. Non-progressive viral myelitis in X-linked agammaglobulinemia. Brain Dev. Mar 2002;24(2):109-11. [Medline].

  19. Tasca G, Iorio R, Basile U, et al. Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia. J Neurol Sci. Sep 15 2009;284(1-2):203-4. [Medline].

  20. Bloom KA, Chung D, Cunningham-Rundles C. Osteoarticular infectious complications in patients with primary immunodeficiencies. Curr Opin Rheumatol. Jul 2008;20(4):480-5. [Medline]. [Full Text].

  21. Bonkowsky JL, Bohnsack JF, Pennington MJ, et al. Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome?. Am J Med Genet A. 2004;128:52-6. [Medline].

  22. Akman S, Guven AG, Ince S, et al. IgG and IgG subclasses deficiency in children undergoing continuous ambulatory peritoneal dialysis and its provocative factors. Pediatr Int. Jun 2002;44(3):273-6. [Medline].

  23. Rehman S, Bytnar D, Berkenbosch JW, et al. Hypogammaglobulinemia in pediatric ICU patients. J Intensive Care Med. Sep-Oct 2003;18(5):261-4. [Medline].

  24. Gulino AV. WHIM syndrome: a genetic disorder of leukocyte trafficking. Curr Opin Allergy Clin Immunol. 2003;3:443-50. [Medline].

  25. Lynn J, Knight AK, Kamoun M, Levinson AI. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts. J Allergy Clin Immunol. Aug 2004;114(2):409-14. [Medline].

  26. Agarwal S, Cunningham-Rundles C. Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. Ann Allergy Asthma Immunol. 2007;98:185-90. [Medline].

  27. Onigbanjo MT, Orange JS, Perez EE, Sullivan KE. Hypogammaglobulinemia in a pediatric tertiary care setting. Clin Immunol. 2007;125:52-9. [Medline].

  28. Sawada A, Takihara Y, Kim JY, et al. A congenital mutation of the novel gene LRRC8 causes agammaglobulinemia in humans. J Clin Invest. Dec 2003;112(11):1707-13. [Medline].

  29. Okumura A, Tsuge I, Kamachi Y et al. Transient hypogammaglobulinemia after antileptic drug hypersensitivity. Pediatr Neurol. 2007;36:342-4. [Medline].

  30. Boccara O, Valevrie-Allanore L, Crickx B et al. Association of hypogammaglobulinemia with DRESS (Drug Rash with Eosinophilia and Systemic Symptoms). Eur J Dermatol. 2006;16:666-8. [Medline]. [Full Text].

  31. Kawano T, Matsuse H, Obase Y, et al. Hypogammaglobulinemia in steroid-dependent asthmatics correlates with the daily dose of oral prednisolone. Int Arch Allergy Immunol. Jul 2002;128(3):240-3. [Medline].

  32. Kitamura A, Takiguchi Y, Tochigi N, et al. Durable hypogammaglobulinemia associated with thymoma (Good syndrome). Intern Med. 2009;48(19):1749-52. [Medline].

  33. Yong PF, Aslam L, Karim MY, Khamashta MA. Management of hypogammaglobulinaemia occurring in patients with systemic lupus erythematosus. Rheumatology (Oxford). Sep 2008;47(9):1400-5. [Medline].

  34. Carneiro-Sampaio M, Liphaus BL, Jesus AA, Silva CA, Oliveira JB, Kiss MH. Understanding systemic lupus erythematosus physiopathology in the light of primary immunodeficiencies. J Clin Immunol. May 2008;28 Suppl 1:S34-41. [Medline].

  35. Orange JS, Geha RS, Bonilla FA. Acute chylothorax in children: selective retention of memory T cells and natural killer cells. J Pediatr. 2003;143:243-9. [Medline].

  36. Bezrodnik L, Raccio AC, Canil LM, Rey MA, Carabajal PC, FOssati CA, et al. Hypogammaglobulinemia secondary to cow-milk allergy in children under 2 years of age. Immunology. 2007;122:140-6. [Medline].

  37. Bondioni MP, Duse M, Plebani A et al. Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed tompgraphy evaluation. J Comput Assist Tompgr. 2007;31:620-8. [Medline].

  38. Rusconi F, Panisi C, Dellepiane RM, et al. Pulmonary and sinus diseases in primary humoral immunodeficiencies with chronic productive cough. Arch Dis Child. Dec 2003;88(12):1101-5. [Medline].

  39. Chinen J, Shearer WT. Subcutaneous immunoglobulins: alternative for the hypogammaglobulinemic patient?. J Allergy Clin Immunol. 2004;114:934-5. [Medline].

  40. Ochs, HD, Gupta S, Kiseeling P et al. Safety and efficacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeficiency diseases. J Clin Immunol. 2006;26:265-73. [Medline].

  41. Gustafson R, Gardulf A, Hansen S et al. Rapid subcutaneous immunoglobulin administration every second week results in high and stable serum immunoglobulin G levels in patients with primary antibody deficiencies. Clin Exp Immunol. 2008;152:274-9. [Medline].

  42. Lacy CF, Armstrong LL, Goldman MP, Lance LL, eds. Drug Information Handbook 2008-2009. 16th edition. Cleveland, OH: Lexi-Comp Inc; 2008.

  43. Hooper JA. Intravenous immunoglobulins: evolution of commercial IVIG preparations. Immunol Allergy Clin North Am. Nov 2008;28(4):765-78, viii. [Medline].

  44. Shah S. Pharmacy considerations for the use of IGIV therapy. Am J Health Syst Pharm. Aug 15 2005;62(16 Suppl 3):S5-11. [Medline].

  45. Siegel J. The Product: All intravenous immunoglobulins are not equivalent. Pharmacother. 2005;25(11 Pt 2):78S-84S.

  46. Howard V, Myers LA, Williams DA, et al. Stem cell transplants for patients with X-linked agammaglobulinemia. Clin Immunol. May 2003;107(2):98-102. [Medline].

  47. Quinti I, Pierdominici M, Marziali M, et al. European surveillance of immunoglobulin safety--results of initial survey of 1243 patients with primary immunodeficiencies in 16 countries. Clin Immunol. Sep 2002;104(3):231-6. [Medline].

  48. Buckley RH. Pulmonary complications of primary immunodeficiencies. Paediatr Respir Rev. 2004;5 (Suppl A):S225-33. [Medline].

  49. Patiroglu T, Akyildiz B, Patiroglu TE, Gulmez IY. Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia. Pediatr Pulmonol. Jul 2008;43(7):710-3. [Medline].

  50. Berlucchi M, Soresina A, Redaelli De Zinis LO, et al. Sensorineural hearing loss in primary antibody deficiency disorders. J Pediatr. Aug 2008;153(2):293-6. [Medline].

  51. Brosens LA, Tytgat KM, Morsink FH et al. Multiple colorectal neoplasms in X-linked agammaglobulinemia. Clin Gastroenterol Hepatol. 2008;6:115-9. [Medline].

  52. Ziegner UH,, Kobayashi RH, Cunningham-Rundles C, et al. Progressive neurodegeneration in patients with primary immunodeficiency disease on IVIG treatment. Clin Immunol. 2002;102:19-24. [Medline].

  53. Papapetropoulos S, Friedman J, Blackstone C et al. A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IBIG therapy. Mov Disord. 2007;22:1664-6. [Medline].

  54. Dalal I, Reid B, Nisbet-Brown E, Roifman CM. The outcome of patients with hypogammaglobulinemia in infancy and early childhood. J Pediatr. Jul 1998;133(1):144-6. [Medline].

  55. Kidon MI, Handzel ZT, Schwartz R, et al. Symptomatic hypogammaglobulinemia in infancy and childhood - clinical outcome and in vitro immune responses. BMC Fam Pract. Oct 21 2004;5:23. [Medline].

  56. Desar IM, Weemaes CM, van Deuren M et al. Reversible hypogammaglubulinaemia. Neth J Med. 2007;65:381-5. [Medline].

  57. Abe S, Suzuki T, Hori T, Baba A, Shiraishi H. Hypogammaglobulinemia during antipsychotic therapy. Psychiatry Clin Neurosci. Feb 1998;52(1):115-7. [Medline].

  58. Aghamohammadi A, Moin M, Farhoudi A, et al. Efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with agammaglobulinemia. FEMS Immunol Med Microbiol. Mar 8 2004;40(2):113-8. [Medline].

  59. Asmar BI, Andresen J, Brown WJ. Ureaplasma urealyticum arthritis and bacteremia in agammaglobulinemia. Pediatr Infect Dis J. Jan 1998;17(1):73-6. [Medline].

  60. Black C, Zavod MB, Gosselin BJ. Haemophilus influenzae lymphadenopathy in a patient with agammaglobulinemia: clinical-histologic-microbiologic correlation and review of the literature. Arch PatholLab Med. 2005;129:100-3. [Medline].

  61. Buehring I, Friedrich B, Schaaf J, et al. Chronic sinusitis refractory to standard management in patients with humoral immunodeficiencies. Clin Exp Immunol. Sep 1997;109(3):468-72. [Medline].

  62. Conley ME. Early defects in B cell development. Curr Opin Allergy Clin Immunol. 2002;2:517-22. [Medline].

  63. Conley ME, Broides A, Hernandez-Trujillo V, et al. Genetic analysis of patients with defects in early B-cell development. Immunol Rev. 2005;203:216-34. [Medline].

  64. Conley ME, Dobbs AK, Farmer DM, et al. Primary B cell immunodeficiencies: comparisons and contrasts. Annu Rev Immunol. 2009;27:199-227. [Medline].

  65. Dittrich AM, Schulze I, Magdorf K, et al. X-linked agammaglobulinaemia and Pneumocystis carinii pneumonia--an unusual coincidence?. Eur J Pediatr. Jun 2003;162(6):432-3. [Medline].

  66. Eijkhout HW, van Der Meer JW, Kallenberg CG, et al. The effect of two different dosages of intravenous immunoglobulin on the incidence of recurrent infections in patients with primary hypogammaglobulinemia. A randomized, double-blind, multicenter crossover trial. Ann Intern Med. Aug 7 2001;135(3):165-74. [Medline].

  67. Feydy A, Sibilia J, De Kerviler E, et al. Chest high resolution CT in adults with primary humoral immunodeficiency. Br J Radiol. Dec 1996;69(828):1108-16. [Medline].

  68. Fijolek J, Wiatr E, Demkow U, Orlowsk TM. Immunological disturbances in Good's syndrome. Clin Invest Med. Aug 1 2009;32(4):E301-6. [Medline].

  69. Halsey NA, Pinto J, Espinosa-Rosales F et al. Search for poliovirus carriers among people with primary immune deficiency diseases in the United States, Mexico, Brazil and the United Kingdom. Bull World Heatlh Organ. 2004;82:3-8. [Medline].

  70. Kainulainen L, Nikoskelainen J, Vuorinen T, et al. Viruses and bacteria in bronchial samples from patients with primary hypogammaglobulinemia. Am J Respir Crit Care Med. Apr 1999;159(4 Pt 1):1199-204. [Medline].

  71. Kano Y, Inaoka M, Shiohara T. Association between anticonvulsant hypersensitivity syndrome and human herpes 6 reactivation and hypogammaglobulinemia. Arch Dermatol. 2004;140:183-8. [Medline].

  72. Kawai T, Malech HL. WHIM syndrome: congenital immune deficiency disease. Curr Opin Hematol. Jan 2009;16(1):20-6. [Medline]. [Full Text].

  73. Kutukculer N, Gulez N. The outcome of patients with unclassified hypogammaglobulinemia in early childhood. Pediatr Allergy Immunol. Nov 2009;20(7):693-8. [Medline].

  74. Morales P, Hernandez D, Vicente R, et al. Lung transplantation in patients with x-linked agammaglobulinemia. Transplant Proc. Aug 2003;35(5):1942-3. [Medline].

  75. Mueller BU, Pizzo PA. Cancer in children with primary or secondary immunodeficiencies. J Pediatr. Jan 1995;126(1):1-10. [Medline].

  76. Ozdoba C, Ramelli G, Schroth G. MRI in a patient with congenital agammaglobulinaemia. Neuroradiology. Aug 1998;40(8):516-8. [Medline].

  77. Plebani A, Soresina A, Rondelli R, et al. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study. Clin Immunol. Sep 2002;104(3):221-30. [Medline].

  78. Quartier P, Debre M, De Blic J, et al. Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients. J Pediatr. May 1999;134(5):589-96. [Medline].

  79. Raynaud M, Ronce N, Ayrault AD, et al. X-linked mental retardation with isolated growth hormone deficiency is mapped to Xq22-Xq27.2 in one family. Am J Med Genet. Mar 19 1998;76(3):255-61. [Medline].

  80. Skull S, Kemp A. Treatment of hypogammaglobulinaemia with intravenous immunoglobulin, 1973-93. Arch Dis Child. Jun 1996;74(6):527-30. [Medline].

  81. Teramoto T, Kaneko H, Funato M, et al. Progressive multifocal leukoencephalopathy in a patient with X-linked agammaglobulinemia. Scand J Infect Dis. 2003;35(11-12):909-10. [Medline].

  82. Tokuda K, Nishi J, Miyanohara H, et al. Relapsing cellulitis associated with Campylobacter coli bacteremia in an agammaglobulinemic patient. Pediatr Infect Dis J. Jun 2004;23(6):577-9. [Medline].

  83. Wang LJ, Yang YH, Lin YT, Chiang BL. Immunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan. Asian Pac J Allergy Immunol. 2004;22:25-31. [Medline].

 
Previous
Next
 
Early stages of B-cell differentiation can be identified by the status of the immunoglobulin genes and by the cell surface markers CD34, CD19, and surface immunoglobulin (sIg). From: Conley ME. Genes required for B cell development. J Clin Invest. 2003;112: 1636-8. Reproduced with permission of American Society for Clinical Investigation via Copyright Clearance Center.
Table 1. Immune Globulin, Intravenous[42, 43, 44, 45]
Brand(Manufacturer)Manufacturing ProcesspHAdditives (IVIG products containing sucrose are more 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 ConcentrationsIgA Content (mcg/mL)
Carimune NF



(ZLB Behring)



Kistler-Nitschmann fractionation; pH 4 incubation, nanofiltration6.4-6.86% solution: 10% sucrose, < 20 mg NaCl/g proteinLyophilized powder 3%, 6%, 9%, 12%Trace
Flebogamma



(Grifols USA)



Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization5.1-6Sucrose free, contains 5% D-sorbitolLiquid 5%< 50
Gammagard Liquid 10%



(Baxter Bioscience)



Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation 4.6-5.10.25M glycineReady-for-use liquid 10%37
Gammar-P IV



(ZLB Behring)



Cohn-Oncley fraction II/III; ultrafiltration; pasteurization6.4-7.25% solution: 5% sucrose, 3% albumin, 0.5% NaClLyophilized powder 5%< 20
Gamunex



(Talecris Biotherapeutics)



Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation4-4.5Contains no sugar, contains glycineLiquid 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 4.8-5.1Contains sorbitol (40 mg/mL); do not administer if fructose intolerantReady-for-use solution 5%< 10
Iveegam EN



(Baxter Bioscience)



Cohn-Oncley fraction II/III; ultrafiltration; pasteurization6.4-7.25% solution: 5% glucose, 0.3% NaClLyophilized 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 6.4-7.25% solution: 0.3% albumin, 2.25% glycine, 2% glucoseLyophilized 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 pasteurization5.1-610% maltoseLiquid 5%200
Panglobulin



(Swiss Red Cross for the American Red Cross)



Kistler-Nitschmann fractionation; pH 4 incubation, trace pepsin, nanofiltration6.6Per gram of IgG: 1.67 g sucrose, < 20 mg NaClLyophilized powder 3%, 6%, 9%, 12%720
Privigen



(CSL Behring)



pH 4 incubation; octanoic acid fractionation, depth filtration, and virus filtration4.6-510% solution; Preservative-free, sucrose-free, and maltose-freeReady-to-use solution 10%< 25
Table 2. Immune Globulin, Subcutaneous
Brand(Manufacturer)Manufacturing ProcesspHAdditivesParenteral Form and Final ConcentrationsIgA Content mcg/mL
Vivaglobin



(ZLB Behring)



Cold ethanol fractionation, pasteurization6.4-7.22.25% glycine, 0.3% NaClLiquid 16% (160 mg/mL)< 50 mcg/mL
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.