Skip to main content
CMAJ : Canadian Medical Association Journal logoLink to CMAJ : Canadian Medical Association Journal
. 1987 Nov 1;137(9):793–797.

Hypogammaglobulinemia: therapeutic rationale.

A A Long 1, J A Denburg 1, P B Dent 1
PMCID: PMC1267348  PMID: 3327579

Abstract

Hypogammaglobulinemia is a feature of several B-cell disorders and is manifested clinically by recurrent infection, most commonly chronic upper and lower respiratory tract disease. Immunoglobulin replacement therapy is available, with at least four different routes of administration. There are as yet no convincing data that allow comparison of the cost-effectiveness of these methods. However, by individualizing therapy for each patient, it is possible to prevent life-threatening acute infections, reduce the severity of chronic upper and lower respiratory tract disease, improve pulmonary function and achieve normal levels of IgG. These are the currently acceptable goals of therapy in patients with hypogammaglobulinemia.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ammann A. J., Ashman R. F., Buckley R. H., Hardie W. R., Krantmann H. J., Nelson J., Ochs H., Stiehm E. R., Tiller T., Wara D. W. Use of intravenous gamma-globulin in antibody immunodeficiency: results of a multicenter controlled trial. Clin Immunol Immunopathol. 1982 Jan;22(1):60–67. doi: 10.1016/0090-1229(82)90022-8. [DOI] [PubMed] [Google Scholar]
  2. Ashida E. R., Saxon A. Home intravenous immunoglobulin therapy by self-administration. J Clin Immunol. 1986 Jul;6(4):306–309. doi: 10.1007/BF00917331. [DOI] [PubMed] [Google Scholar]
  3. BARANDUN S., KISTLER P., JEUNET F., ISLIKER H. Intravenous administration of human gamma-globulin. Vox Sang. 1962;7:157–174. doi: 10.1111/j.1423-0410.1962.tb03240.x. [DOI] [PubMed] [Google Scholar]
  4. BRUTON O. C. Agammaglobulinemia. Pediatrics. 1952 Jun;9(6):722–728. [PubMed] [Google Scholar]
  5. Ben-Bassat I., Many A., Modan M., Peretz C., Ramot B. Serum immunoglobulins in chronic lymphocytic leukemia. Am J Med Sci. 1979 Jul-Aug;278(1):4–9. doi: 10.1097/00000441-197907000-00001. [DOI] [PubMed] [Google Scholar]
  6. Berger M., Cupps T. R., Fauci A. S. Immunoglobulin replacement therapy by slow subcutaneous infusion. Ann Intern Med. 1980 Jul;93(1):55–56. doi: 10.7326/0003-4819-93-1-55. [DOI] [PubMed] [Google Scholar]
  7. Besa E. C. Use of intravenous immunoglobulin in chronic lymphocytic leukemia. Am J Med. 1984 Mar 30;76(3A):209–218. doi: 10.1016/0002-9343(84)90344-9. [DOI] [PubMed] [Google Scholar]
  8. Björkander J., Bake B., Hanson L. A. Primary hypogammaglobulinaemia: impaired lung function and body growth with delayed diagnosis and inadequate treatment. Eur J Respir Dis. 1984 Oct;65(7):529–536. [PubMed] [Google Scholar]
  9. Broder S., Humphrey R., Durm M., Blackman M., Meade B., Goldman C., Strober W., Waldmann T. Impaired synthesis of polyclonal (non-paraprotein) immunoglobulins by circulating lymphocytes from patients with multiple myeloma Role of suppressor cells. N Engl J Med. 1975 Oct 30;293(18):887–892. doi: 10.1056/NEJM197510302931801. [DOI] [PubMed] [Google Scholar]
  10. Buckley R. H. Immunodeficiency. J Allergy Clin Immunol. 1983 Dec;72(6):627–641. doi: 10.1016/0091-6749(83)90619-x. [DOI] [PubMed] [Google Scholar]
  11. Cunningham-Rundles C., Siegal F. P., Smithwick E. M., Lion-Boulé A., Cunningham-Rundles S., O'Malley J., Barandun S., Good R. A. Efficacy of intravenous immunoglobulin in primary humoral immunodeficiency disease. Ann Intern Med. 1984 Oct;101(4):435–439. doi: 10.7326/0003-4819-101-4-435. [DOI] [PubMed] [Google Scholar]
  12. Dwyer J. M. Thirty years of supplying the missing link. History of gamma globulin therapy for immunodeficient states. Am J Med. 1984 Mar 30;76(3A):46–52. doi: 10.1016/0002-9343(84)90319-x. [DOI] [PubMed] [Google Scholar]
  13. Eibl M. Treatment of defects of humoral immunity. Birth Defects Orig Artic Ser. 1983;19(3):193–200. [PubMed] [Google Scholar]
  14. Gale R. P., Foon K. A. Chronic lymphocytic leukemia. Recent advances in biology and treatment. Ann Intern Med. 1985 Jul;103(1):101–120. doi: 10.7326/0003-4819-103-1-101. [DOI] [PubMed] [Google Scholar]
  15. Geha R. S., Schneeberger E., Merler E., Rosen F. S. Heterogeneity of "acquired" or common variable agammaglobulinemia. N Engl J Med. 1974 Jul 4;291(1):1–6. doi: 10.1056/NEJM197407042910101. [DOI] [PubMed] [Google Scholar]
  16. Gordon D. S., Hearn E. B., Spira T. J., Reimer C. B., Phillips D. J., Schable C. Phase I study of intravenous gamma globulin in multiple myeloma. Am J Med. 1984 Mar 30;76(3A):111–116. doi: 10.1016/0002-9343(84)90328-0. [DOI] [PubMed] [Google Scholar]
  17. Hayakawa H., Iwata T., Yata J., Kobayashi N. Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol. 1981 Jan;1(1):31–39. doi: 10.1007/BF00915474. [DOI] [PubMed] [Google Scholar]
  18. Huser H. J., Schwander D., Wegmann A., Chariatte N., Fässler M. A. Verträglichkeit und Verweildauer eines intravenösen Immunglobulinpräparates bei immunologisch gesunden Personen und Verträglichkeit bei Patienten mit Hypogammaglobulinämie infolge chronischer lymphatischer Leukämie. Schweiz Med Wochenschr. 1986 Feb 1;116(5):151–156. [PubMed] [Google Scholar]
  19. JANEWAY C. A., APT L., GITLIN D. Agammaglobulinemia. Trans Assoc Am Physicians. 1953;66:200–202. [PubMed] [Google Scholar]
  20. Janeway C. A., Rosen F. S. The gamma globulins. IV. Therapeutic uses of gamma globulin. N Engl J Med. 1966 Oct 13;275(15):826–831. doi: 10.1056/NEJM196610132751508. [DOI] [PubMed] [Google Scholar]
  21. Koch C., Andersen V., Faber V., Friis B., Pedersen F. K., Platz P. Primaere immundefekter i Danmark. Rapport fra Dansk register for primaere immundefekter. Ugeskr Laeger. 1981 Sep 21;143(39):2479–2484. [PubMed] [Google Scholar]
  22. Lederman H. M., Roifman C. M., Lavi S., Gelfand E. W. Corticosteroids for prevention of adverse reactions to intravenous immune serum globulin infusions in hypogammaglobulinemic patients. Am J Med. 1986 Sep;81(3):443–446. doi: 10.1016/0002-9343(86)90296-2. [DOI] [PubMed] [Google Scholar]
  23. Luzi G., Businco L., Aiuti F. A national registry for primary immunodeficiency syndromes in Italy: a report for the period 1972-1982. Birth Defects Orig Artic Ser. 1983;19(3):161–163. [PubMed] [Google Scholar]
  24. Nolte M. T., Pirofsky B., Gerritz G. A., Golding B. Intravenous immunoglobulin therapy for antibody deficiency. Clin Exp Immunol. 1979 May;36(2):237–243. [PMC free article] [PubMed] [Google Scholar]
  25. Ochs H. D., Fischer S. H., Lee M. L., Delson E. S., Kingdon H. S., Wedgwood R. J. Intravenous immunoglobulin home treatment for patients with primary immunodeficiency diseases. Lancet. 1986 Mar 15;1(8481):610–611. doi: 10.1016/s0140-6736(86)92826-6. [DOI] [PubMed] [Google Scholar]
  26. Ochs H. D., Fischer S. H., Wedgwood R. J., Wara D. W., Cowan M. J., Ammann A. J., Saxon A., Budinger M. D., Allred R. U., Rousell R. H. Comparison of high-dose and low-dose intravenous immunoglobulin therapy in patients with primary immunodeficiency diseases. Am J Med. 1984 Mar 30;76(3A):78–82. doi: 10.1016/0002-9343(84)90324-3. [DOI] [PubMed] [Google Scholar]
  27. Reinherz E. L., Rubinstein A., Geha R. S., Strelkauskas A. J., Rosen F. S., Schlossman S. F. Abnormalities of immunoregulatory T cells in disorders of immune function. N Engl J Med. 1979 Nov 8;301(19):1018–1022. doi: 10.1056/NEJM197911083011902. [DOI] [PubMed] [Google Scholar]
  28. Roifman C. M., Lederman H. M., Lavi S., Stein L. D., Levison H., Gelfand E. W. Benefit of intravenous IgG replacement in hypogammaglobulinemic patients with chronic sinopulmonary disease. Am J Med. 1985 Aug;79(2):171–174. doi: 10.1016/0002-9343(85)90006-3. [DOI] [PubMed] [Google Scholar]
  29. Roifman C. M., Levison H., Gelfand E. W. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet. 1987 May 9;1(8541):1075–1077. doi: 10.1016/s0140-6736(87)90494-6. [DOI] [PubMed] [Google Scholar]
  30. Rosen F. S., Cooper M. D., Wedgwood R. J. The primary immunodeficiencies. (2). N Engl J Med. 1984 Aug 2;311(5):300–310. doi: 10.1056/NEJM198408023110506. [DOI] [PubMed] [Google Scholar]
  31. Salmon S. E., Samal B. A., Hayes D. M., Hosley H., Miller S. P., Schilling A. Role of gamma globulin for immunoprophylaxis in multiple myeloma. N Engl J Med. 1967 Dec 21;277(25):1336–1340. doi: 10.1056/NEJM196712212772503. [DOI] [PubMed] [Google Scholar]
  32. Schiff R. I., Rudd C., Johnson R., Buckley R. H. Individualization of gamma globulin dosage in patients with humoral immunodeficiency. Birth Defects Orig Artic Ser. 1983;19(3):209–212. [PubMed] [Google Scholar]
  33. Stiehm E. R., Vaerman J. P., Fudenberg H. H. Plasma infusions in immunologic deficiency states: metabolic and therapeutic studies. Blood. 1966 Dec;28(6):918–937. [PubMed] [Google Scholar]
  34. Ugazio A. G., Duse M., Re R., Mangili G., Burgio G. R. Subcutaneous infusion of gammaglobulins in management of agammaglobulinaemia. Lancet. 1982 Jan 23;1(8265):226–226. doi: 10.1016/s0140-6736(82)90793-0. [DOI] [PubMed] [Google Scholar]
  35. Waldmann T. A., Durm M., Broder S., Blackman M., Blaese R. M., Strober W. Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinaemia. Lancet. 1974 Sep 14;2(7881):609–613. doi: 10.1016/s0140-6736(74)91940-0. [DOI] [PubMed] [Google Scholar]
  36. de la Concha E. G., Oldham G., Webster A. D., Asherson G. L., Platts-Mills T. A. Quantitative measurements of T- and B-cell function in "variable" primary hypogammaglobulinaemia: evidence for a consistent B-cell defect. Clin Exp Immunol. 1977 Feb;27(2):208–215. [PMC free article] [PubMed] [Google Scholar]

Articles from CMAJ: Canadian Medical Association Journal are provided here courtesy of Canadian Medical Association

RESOURCES