Skip to main content
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1987 Jun;68(3):630–637.

Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes.

T Ohno 1, M Inaba 1, K Kuribayashi 1, T Masuda 1, T Kanoh 1, H Uchino 1
PMCID: PMC1542763  PMID: 2958191

Abstract

Peripheral blood lymphocytes from four patients with selective IgM deficiency were examined phenotypically and functionally. Although B cell subpopulations determined by surface immunoglobulins were within normal or nearly normal range, T8+ cells were significantly increased and T4/T8 ratios were inverted in three patients. IgM specific hyporesponsiveness in the PWM-driven immunoglobulin production system was observed in all four patients. Ia-like antigen positive T cells were increased in two patients; both had increased Leu2a+ Leu15+ suppressor-effector cells. In addition, Leu3a+ Leu8+ suppressor-inducer cells were increased in one of these two patients. Excessive (either IgM-specific or isotype non-specific) suppressor activity of T cells and IgM specific hyporesponsiveness of non-T cells were observed in these two patients in the recombination plaque assay. Although these results showed the complexity of the pathogenesis of this syndrome, they suggested that suppressor-associated T cells may play a role in some patients with selective IgM deficiency.

Full text

PDF
631

Selected References

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

  1. Ammann A. J., Wara D. W., Pillarisetty R. J., Talal N. The prevalence of autoantibodies in T-cell, B-cell, and phagocytic immunodeficiency disorders. Clin Immunol Immunopathol. 1979 Dec;14(4):456–466. doi: 10.1016/0090-1229(79)90098-9. [DOI] [PubMed] [Google Scholar]
  2. Clement L. T., Grossi C. E., Gartland G. L. Morphologic and phenotypic features of the subpopulation of Leu-2+ cells that suppresses B cell differentiation. J Immunol. 1984 Nov;133(5):2461–2468. [PubMed] [Google Scholar]
  3. Damle N. K., Mohagheghpour N., Engleman E. G. Soluble antigen-primed inducer T cells activate antigen-specific suppressor T cells in the absence of antigen-pulsed accessory cells: phenotypic definition of suppressor-inducer and suppressor-effector cells. J Immunol. 1984 Feb;132(2):644–650. [PubMed] [Google Scholar]
  4. De la Concha E. G., Garcia-Rodriguez M. C., Zabay J. M., Laso M. T., Alonso F., Bootello A., Fontan G. Functional assessment of T and B lymphocytes in patients with selective IgM deficiency. Clin Exp Immunol. 1982 Sep;49(3):670–676. [PMC free article] [PubMed] [Google Scholar]
  5. Dworzack D. L., Murray C. M., Hodges G. R., Barnes W. G. Community-acquired bacteremic Achromobacter xylosoxidans type IIIa pneumonia in a patient with idiopathic IgM deficiency. Am J Clin Pathol. 1978 Oct;70(4):712–717. doi: 10.1093/ajcp/70.4.712. [DOI] [PubMed] [Google Scholar]
  6. FUDENBERG H., GERMAN J. L., 3rd, KUNKEL H. G. The occurrence of rheumatoid factor and other abnormalities in families of patients with agammaglobulinemia. Arthritis Rheum. 1962 Dec;5:565–588. doi: 10.1002/art.1780050605. [DOI] [PubMed] [Google Scholar]
  7. Faulk W. P., Kiyasu W. S., Cooper M. D., Fudenberg H. H. Deficiency of IgM. Pediatrics. 1971 Feb;47(2):399–404. [PubMed] [Google Scholar]
  8. Gatenby P. A., Kansas G. S., Xian C. Y., Evans R. L., Engleman E. G. Dissection of immunoregulatory subpopulations of T lymphocytes within the helper and suppressor sublineages in man. J Immunol. 1982 Nov;129(5):1997–2000. [PubMed] [Google Scholar]
  9. Gronowicz E., Coutinho A., Melchers F. A plaque assay for all cells secreting Ig of a given type or class. Eur J Immunol. 1976 Aug;6(8):588–590. doi: 10.1002/eji.1830060812. [DOI] [PubMed] [Google Scholar]
  10. Hermans P. E., Diaz-Buxo J. A., Stobo J. D. Idiopathic late-onset immunoglobulin deficiency. Clinical observations in 50 patients. Am J Med. 1976 Aug;61(2):221–237. doi: 10.1016/0002-9343(76)90173-x. [DOI] [PubMed] [Google Scholar]
  11. Hobbs J. R., Milner R. D., Watt P. J. Gamma-M deficiency predisposing to meningococcal septicaemia. Br Med J. 1967 Dec 9;4(5579):583–586. doi: 10.1136/bmj.4.5579.583. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Jackson R. A., Morris M. A., Haynes B. F., Eisenbarth G. S. Increased circulating Ia-antigen-bearing T cells in type I diabetes mellitus. N Engl J Med. 1982 Apr 1;306(13):785–788. doi: 10.1056/NEJM198204013061305. [DOI] [PubMed] [Google Scholar]
  13. Jones D. M., Tobin B. M., Butterworth A. Three cases of meningococcal infection in a family, associated with a deficient immune response. Arch Dis Child. 1973 Sep;48(9):742–743. doi: 10.1136/adc.48.9.742. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Kaufman H. S., Hobbs J. R. Immunoglobulin deficiencies in an atopic population. Lancet. 1970 Nov 21;2(7682):1061–1063. doi: 10.1016/s0140-6736(70)90288-6. [DOI] [PubMed] [Google Scholar]
  15. Kersey J. H., Spector B. D., Good R. A. Cancer in children with primary immunodeficiency diseases. J Pediatr. 1974 Feb;84(2):263–264. doi: 10.1016/s0022-3476(74)80619-0. [DOI] [PubMed] [Google Scholar]
  16. Ko H. S., Fu S. M., Winchester R. J., Yu D. T., Kunkel H. G. Ia determinants on stimulated human T lymphocytes. Occurrence on mitogen- and antigen-activated T cells. J Exp Med. 1979 Aug 1;150(2):246–255. doi: 10.1084/jem.150.2.246. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Kuritani T., Cooper M. D. Human B cell differentiation. II. Pokeweed mitogen-responsive B cells belong to a surface immunoglobulin D-negative subpopulation. J Exp Med. 1982 May 1;155(5):1561–1566. doi: 10.1084/jem.155.5.1561. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Landay A., Gartland G. L., Clement L. T. Characterization of a phenotypically distinct subpopulation of Leu-2+ cells that suppresses T cell proliferative responses. J Immunol. 1983 Dec;131(6):2757–2761. [PubMed] [Google Scholar]
  19. Leung D. Y., Chu E. T., Wood N., Grady S., Meade R., Geha R. S. Immunoregulatory T cell abnormalities in mucocutaneous lymph node syndrome. J Immunol. 1983 May;130(5):2002–2004. [PubMed] [Google Scholar]
  20. Mayer L., Kwan S. P., Thompson C., Ko H. S., Chiorazzi N., Waldmann T., Rosen F. Evidence for a defect in "switch" T cells in patients with immunodeficiency and hyperimmunoglobulinemia M. N Engl J Med. 1986 Feb 13;314(7):409–413. doi: 10.1056/NEJM198602133140703. [DOI] [PubMed] [Google Scholar]
  21. Mayer L., Posnett D. N., Kunkel H. G. Human malignant T cells capable of inducing an immunoglobulin class switch. J Exp Med. 1985 Jan 1;161(1):134–144. doi: 10.1084/jem.161.1.134. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Reinherz E. L., Kung P. C., Pesando J. M., Ritz J., Goldstein G., Schlossman S. F. Ia determinants on human T-cell subsets defined by monoclonal antibody. Activation stimuli required for expression. J Exp Med. 1979 Dec 1;150(6):1472–1482. doi: 10.1084/jem.150.6.1472. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Saxon A., Feldhaus J., Robins R. A. Single step separation of human T and B cells using AET treated srbc rosettes. J Immunol Methods. 1976;12(3-4):285–288. doi: 10.1016/0022-1759(76)90050-8. [DOI] [PubMed] [Google Scholar]
  24. Seki H., Taga K., Nagaoki T., Barot-Ciorbaru R., Miyawaki T., Taniguchi N. Some evidence for the in vivo functional activation of suppressor T cells in asymptomatic patients with hemophilia A receiving Factor VIII concentrates. Clin Immunol Immunopathol. 1985 Jan;34(1):27–38. doi: 10.1016/0090-1229(85)90004-2. [DOI] [PubMed] [Google Scholar]
  25. Siegal F. P., Siegal M. Enhancement by irradiated T cells of human plasma cell production: dissection of helper and suppressor functions in vitro. J Immunol. 1977 Feb;118(2):642–647. [PubMed] [Google Scholar]
  26. Stoelinga G. B., van Munster P. J. Antibody deficiency syndrome and autoimmune haemolytic anaemia in a boy with isolated IgM deficiency dysimmunoglobulinaemia type 5. Acta Paediatr Scand. 1969 Jul;58(4):352–362. doi: 10.1111/j.1651-2227.1969.tb04731.x. [DOI] [PubMed] [Google Scholar]
  27. Takenaka T., Nakamine H., Nishihara T., Tsuda T., Tsujimoto M., Maeda J. Prolymphocytic leukemia with IgM hypogammaglobulinemia. Am J Clin Pathol. 1983 Aug;80(2):237–242. doi: 10.1093/ajcp/80.2.237. [DOI] [PubMed] [Google Scholar]
  28. Vogelzang N. J., Corwin H., Finlay J. L., Pellettiere E. V., 2nd, Luskin A. T., Di Camelli R. F., Hong R. Clear cell sarcoma and selective IgM deficiency: a case report. Cancer. 1982 Jan 15;49(2):234–238. doi: 10.1002/1097-0142(19820115)49:2<234::aid-cncr2820490207>3.0.co;2-h. [DOI] [PubMed] [Google Scholar]
  29. Williams R. C., Jr, Webster A. D., Morito T., Greaves M. F. T lymphocyte subpopulations and Ia-positive T cells in patients with immunodeficiency. Clin Exp Immunol. 1980 Nov;42(2):355–363. [PMC free article] [PubMed] [Google Scholar]
  30. Yachie A., Miyawaki T., Yokoi T., Nagaoki T., Taniguchi N. Ia-positive cells generated by PWM-stimulation within OKT4+ subset interact with OKT8+ cells for inducing active suppression on B cell differentiation in vitro. J Immunol. 1982 Jul;129(1):103–106. [PubMed] [Google Scholar]
  31. Yu D. T., Winchester R. J., Fu S. M., Gibofsky A., Ko H. S., Kunkel H. G. Peripheral blood Ia-positive T cells. Increases in certain diseases and after immunization. J Exp Med. 1980 Jan 1;151(1):91–100. doi: 10.1084/jem.151.1.91. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Clinical and Experimental Immunology are provided here courtesy of British Society for Immunology

RESOURCES