Skip to main content
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1968 Oct;3(8):843–856.

Lymphocytes in congenital immunological deficiency diseases

S P Gotoff
PMCID: PMC1578970  PMID: 5752744

Abstract

The in vitro lymphocyte response to phytohaemagglutinin (PHA) and antigens was normal in five patients with Bruton-type agammaglobulinaemia. Lymphocytes from one infant with thymic dysplasia failed to respond to PHA. The response to PHA and antigens was variable in three patients with ataxia telangiectasia. The in vitro lymphocyte response in these patients appears to reflect the state of thymic-dependent cellular immunity or capacity for delayed hypersensitivity reactions.

The in vitro lymphocyte response to PHA and antigens was normal in two children with Wiskott-Aldrich Syndrome, a disease in which the immunological defect remains controversial. Lymphocytes from a child who had been thymectomized at birth responded normally to PHA, and a normal capacity for delayed hypersensitivity reactions was demonstrated by skin test and homograft rejection.

Full text

PDF
843

Images in this article

Selected References

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

  1. ALDRICH R. A., STEINBERG A. G., CAMPBELL D. C. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. Pediatrics. 1954 Feb;13(2):133–139. [PubMed] [Google Scholar]
  2. BACH F., HIRSCHHORN K. LYMPHOCYTE INTERACTION: A POTENTIAL HISTOCOMPATIBILITY TEST IN VITRO. Science. 1964 Feb 21;143(3608):813–814. doi: 10.1126/science.143.3608.813. [DOI] [PubMed] [Google Scholar]
  3. BAIN B., LOWENSTEIN L. GENETIC STUDIES ON THE MIXED LEUKOCYTE REACTION. Science. 1964 Sep 18;145(3638):1315–1316. doi: 10.1126/science.145.3638.1315. [DOI] [PubMed] [Google Scholar]
  4. BODER E., SEDGWICK R. P. Ataxia-telangiectasia; a familial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent pulmonary infection. Pediatrics. 1958 Apr;21(4):526–554. [PubMed] [Google Scholar]
  5. BRUTON O. C. Agammaglobulinemia. Pediatrics. 1952 Jun;9(6):722–728. [PubMed] [Google Scholar]
  6. Bach F. H., Voynow N. K. One-way stimulation in mixed leukocyte cultures. Science. 1966 Jul 29;153(3735):545–547. doi: 10.1126/science.153.3735.545. [DOI] [PubMed] [Google Scholar]
  7. Bradley J., Oppenheim J. J. The in vitro proliferation of lymphocytes from patients with hypogammaglobulinaemia. Clin Exp Immunol. 1967 Sep;2(5):549–557. [PMC free article] [PubMed] [Google Scholar]
  8. Epstein W. L., Fudenberg H. H., Reed W. B., Boder E., Sedgwick R. P. Immunologic studies in ataxia-telangiectasia. I. Delayed hypersensitivity and serum immune globulin levels in probands and first-degree relatives. Int Arch Allergy Appl Immunol. 1966;30(1):15–29. [PubMed] [Google Scholar]
  9. FIREMAN P., BOESMAN M., GITLIN D. ATAXIA TELANGIECTASIS. A DYSGAMMAGLOBULINAEMIA WITH DEFICIENT GAMMA-1-A (BETA-2-A)-GLOBULIN. Lancet. 1964 May 30;1(7344):1193–1195. doi: 10.1016/s0140-6736(64)91210-3. [DOI] [PubMed] [Google Scholar]
  10. FUDENBERG H. H., HIRSCHHORN K. AGAMMAGLOBULINEMIA: THE FUNDAMENTAL DEFECT. Science. 1964 Aug 7;145(3632):611–612. doi: 10.1126/science.145.3632.611. [DOI] [PubMed] [Google Scholar]
  11. Fellner M. J., Baer R. L., Ripps C. S., Hirschhorn K. Response of lymphocytes to penicillin: comparison with skin tests and circulating antibodies in man. Nature. 1967 Nov 25;216(5117):803–804. doi: 10.1038/216803a0. [DOI] [PubMed] [Google Scholar]
  12. Forbes I. J., Turner K. J. Synthesis of protein by human lymphocytes in vitro. I. Clinical studies. Australas Ann Med. 1965 Nov;14(4):304–310. doi: 10.1111/imj.1965.14.4.304. [DOI] [PubMed] [Google Scholar]
  13. GOOD R. A., KELLY W. D., ROTSTEIN J., VARCO R. L. Immunological deficiency diseases. Agammaglobulinemia, hypogammaglobulinemia, Hodgkin's disease and sarcoidosis. Prog Allergy. 1962;6:187–319. [PubMed] [Google Scholar]
  14. GOWANS J. L. The recirculation of lymphocytes from blood to lymph in the rat. J Physiol. 1959 Apr 23;146(1):54–69. doi: 10.1113/jphysiol.1959.sp006177. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Gotoff S. P., Amirmokri E., Liebner E. J. Ataxia telangiectasia. Neoplasia, untoward response to x-irradiation, and tuberous sclerosis. Am J Dis Child. 1967 Dec;114(6):617–625. doi: 10.1001/archpedi.1967.02090270073006. [DOI] [PubMed] [Google Scholar]
  16. Gotoff S. P. Lymphocytes in agammaglobulinemia: in vitro response to a specific antigen. Pediatr Res. 1968 May;2(3):209–214. doi: 10.1203/00006450-196805000-00008. [DOI] [PubMed] [Google Scholar]
  17. HIRSCHHORN K., BACH F., KOLODNY R. L., FIRSCHEIN I. L., HASHEM N. IMMUNE RESPONSE AND MITOSIS OF HUMAN PERIPHERAL BLOOD LYMPHOCYTES IN VITRO. Science. 1963 Nov 29;142(3596):1185–1187. doi: 10.1126/science.142.3596.1185. [DOI] [PubMed] [Google Scholar]
  18. HIRSCHHORN K., SCHREIBMAN R. R., BACH F. H., SILTZBACH L. E. IN-VITRO STUDIES OF LYMPHOCYTES FROM PATIENTS WITH SARCOIDOSIS AND LYMPHOPROLIFERATIVE DISEASES. Lancet. 1964 Oct 17;2(7364):842–843. doi: 10.1016/s0140-6736(64)90691-9. [DOI] [PubMed] [Google Scholar]
  19. HITZIG W. H., BIRO Z., BOSCH H., HUSER H. J. Agammaglobulinämie und Alymphocytose mit Schwund des lymphatischen Gewebes. Helv Paediatr Acta. 1958 Dec;13(6):551–585. [PubMed] [Google Scholar]
  20. Hersh E. M., Oppenheim J. J. Impaired in vitro lymphocyte transformation in Hodgkin's disease. N Engl J Med. 1965 Nov 4;273(19):1006–1012. doi: 10.1056/NEJM196511042731903. [DOI] [PubMed] [Google Scholar]
  21. KRIVIT W., GOOD R. A. Aldrich's syndrome (thrombocytopenia, eczema, and infection infants); studies of the defense mechanisms. AMA J Dis Child. 1959 Feb;97(2):137–153. doi: 10.1001/archpedi.1959.02070010139001. [DOI] [PubMed] [Google Scholar]
  22. LANDY M., SANDERSON R. P., BERNSTEIN M. T., JACKSON A. L. ANTIBODY PRODUCTION BY LEUCOCYTES IN PERIPHERAL BLOOD. Nature. 1964 Dec 26;204:1320–1321. doi: 10.1038/2041320a0. [DOI] [PubMed] [Google Scholar]
  23. LING N. R., SOOTHILL J. F. LYMPHOCYTIC TRANSFORMATION. Br Med J. 1964 Dec 5;2(5422):1460–1461. doi: 10.1136/bmj.2.5422.1460-e. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Leikin S. L., Bazelon M., Park K. H. In vitro lymphocyte transformation in ataxia telangiectasia. J Pediatr. 1966 Mar;68(3):477–479. doi: 10.1016/s0022-3476(66)80255-x. [DOI] [PubMed] [Google Scholar]
  25. MILLER J. F. Immunological function of the thymus. Lancet. 1961 Sep 30;2(7205):748–749. doi: 10.1016/s0140-6736(61)90693-6. [DOI] [PubMed] [Google Scholar]
  26. MOYNIHAN P. C., JACKSON J. F., HARDY J. D. LYMPHOCYTE TRANSFORMATION AS AN IN-VITRO HISTOCOMPATIBILITY TEST. Lancet. 1965 Feb 27;1(7383):453–455. doi: 10.1016/s0140-6736(65)91587-4. [DOI] [PubMed] [Google Scholar]
  27. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  28. McFarland W., Heilman D. H. Comparison of lymphocyte transformation and intradermal reactions to tuberculins. Am Rev Respir Dis. 1966 May;93(5):742–748. doi: 10.1164/arrd.1966.93.5.742. [DOI] [PubMed] [Google Scholar]
  29. Meuwissen H. J., Bach F. H., Hong R., Good R. A. Lymphocyte studies in congenital thymic dysplasia: The one-way stimulation test. J Pediatr. 1968 Feb;72(2):177–185. doi: 10.1016/s0022-3476(68)80306-3. [DOI] [PubMed] [Google Scholar]
  30. Miller M. E. Thymic dysplasia ("Swiss agammaglobulinemia"). I. Graft versus host reaction following bone-marrow transfusion. J Pediatr. 1967 May;70(5):730–736. doi: 10.1016/s0022-3476(67)80323-8. [DOI] [PubMed] [Google Scholar]
  31. Mills J. A. The immunologic significance of antigen induced lymphocyte transformation in vitro. J Immunol. 1966 Aug;97(2):239–247. [PubMed] [Google Scholar]
  32. Olson G. B., South M. A., Good R. A. Phytohaemagglutinin unresponsiveness of lymphocytes from babies with congenital rubella. Nature. 1967 May 13;214(5089):695–696. doi: 10.1038/214695a0. [DOI] [PubMed] [Google Scholar]
  33. Oppenheim J. J., Barlow M., Waldmann T. A., Block J. B. Impaired in vitro lymphocyte transformation in patients with ataxia-telangiectasis. Br Med J. 1966 Aug 6;2(5509):330–333. doi: 10.1136/bmj.2.5509.330. [DOI] [PMC free article] [PubMed] [Google Scholar]
  34. Oppenheim J. J., Wolstencroft R. A., Gell P. G. Delayed hypersensitivity in the guinea-pig to a protein-hapten conjugate and its relationship to in vitro transformation of lymph node, spleen, thymus and peripheral blood lymphocytes. Immunology. 1967 Jan;12(1):89–102. [PMC free article] [PubMed] [Google Scholar]
  35. PEARMAIN G., LYCETTE R. R., FITZGERALD P. H. Tuberculin-induced mitosis in peripheral blood leucocytes. Lancet. 1963 Mar 23;1(7282):637–638. doi: 10.1016/s0140-6736(63)91275-3. [DOI] [PubMed] [Google Scholar]
  36. PETERSON R. D., COOPER M. D., GOOD R. A. THE PATHOGENESIS OF IMMUNOLOGIC DEFICIENCY DISEASES. Am J Med. 1965 Apr;38:579–604. doi: 10.1016/0002-9343(65)90135-x. [DOI] [PubMed] [Google Scholar]
  37. PETERSON R. D., KELLY W. D., GOOD R. A. ATAXIA-TELANGIECTASIA. ITS ASSOCIATION WITH A DEFECTIVE THYMUS, IMMUNOLOGICAL-DEFICIENCY DISEASE, AND MALIGNANCY. Lancet. 1964 May 30;1(7344):1189–1193. doi: 10.1016/s0140-6736(64)91209-7. [DOI] [PubMed] [Google Scholar]
  38. Peterson R. D., Cooper M. D., Good R. A. Lymphoid tissue abnormalities associated with ataxia-telangiectasia. Am J Med. 1966 Sep;41(3):342–359. doi: 10.1016/0002-9343(66)90080-5. [DOI] [PubMed] [Google Scholar]
  39. Rosen F. S., Gotoff S. P., Craig J. M., Ritchie J., Janeway C. A. Further observations on the Swiss type of agammaglobulinemia (alymphocytosis). The effect of syngeneic bone-marrow cells. N Engl J Med. 1966 Jan 6;274(1):18–21. doi: 10.1056/NEJM196601062740104. [DOI] [PubMed] [Google Scholar]
  40. Rosen F. S., Janeway C. A. The gamma globulins. 3. The antibody deficiency syndromes. N Engl J Med. 1966 Oct 6;275(14):769–concl. doi: 10.1056/NEJM196610062751407. [DOI] [PubMed] [Google Scholar]
  41. Sell S., Rowe D. S., Gell P. G. Studies on rabbit lymphocytes in vitro. 3. Proteins, RNA, and DNA synthesis by lymphocyte cultures after stimulation with phytohaemagglutinin, with staphylococcal filtrate, with antiallotype serum, and with heterologous antiserum to rabbit whole serum. J Exp Med. 1965 Oct 1;122(4):823–839. doi: 10.1084/jem.122.4.823. [DOI] [PMC free article] [PubMed] [Google Scholar]
  42. Stiehm E. R., McIntosh R. M. Wiskott-Aldrich syndrome: review and report of a large family. Clin Exp Immunol. 1967 Mar;2(2):179–189. [PMC free article] [PubMed] [Google Scholar]
  43. TOBLER R., COTTIER H. Familiäre Lymphopenie mit Agammaglobulinämie und schwerer Moniliasis: die essentielle Lymphocytophthise als besondere Form der frühkindlichen Agammaglobulinamie. Helv Paediatr Acta. 1958 Oct;13(4):313–338. [PubMed] [Google Scholar]
  44. Wolff J. A. Wiskott-Aldrich syndrome: clinical, immunologic, and pathologic observations. J Pediatr. 1967 Feb;70(2):221–232. doi: 10.1016/s0022-3476(67)80417-7. [DOI] [PubMed] [Google Scholar]
  45. van Furth R., Schuit H. R., Hijmans W. The formation of immunoglobulins by human tissues in vitro. IV. Circulating lymphocytes in normal and pathological conditions. Immunology. 1966 Jul;11(1):29–40. [PMC free article] [PubMed] [Google Scholar]

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

RESOURCES