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. 1967 Sep;2(5):589–599.

Dysgammaglobulinaemia type IV C

J R Hobbs, A Russell, Sheila M Worlledge
PMCID: PMC1578786  PMID: 4168878

Abstract

Two further patients with isolated deficiency of serum γA-globulin, low normal γG-globulin and increased γM-globulin are described. Following antigenic challenge very little antibody activity could be detected in their sera. No defect of cellular immunity was shown.

Associated autoimmune haemolytic anaemia and thrombocytopenic purpura were improved by corticosteroid therapy and in one case by splenectomy.

The immunoglobulin pattern is unusual in adults and may be diagnostic of dysgammaglobulinaemia.

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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. BACHMANN R., LAURELL C. B. STUDIES ON THE SERUM GAMMA 1A-GLOBULIN LEVEL. I. HEALTHY SUBJECTS AND HYPERGAMMAGLOBULINEMIC PATIENTS. Scand J Clin Lab Invest. 1965;17:39–45. doi: 10.3109/00365516509077281. [DOI] [PubMed] [Google Scholar]
  3. BARANDUN S., STAMPFLI K., SPENGLER G. A., RIVA G. Die Klinik des Antikörpermangelsyndroms. Helv Med Acta. 1959 May;26(2):163–contd. [PubMed] [Google Scholar]
  4. BURTIN P., BUFFE D., GRABAR P. LES HYPOGAMMAGLOBULIN'EMIES ATYPIQUES (HYPOGAMMAGLOBULIN'EMIES R'ECESSIVES NON LI'EES AU SEXE). ETUDE IMMUNOCHIMIQUE ET G'EN'ETIQUE. Ann Inst Pasteur (Paris) 1964 Apr;106:519–542. [PubMed] [Google Scholar]
  5. CITRON K. M. Agammaglobulinaemia with splenomegaly. Br Med J. 1957 May 18;1(5028):1148–1151. doi: 10.1136/bmj.1.5028.1148. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Crabbé P. A., Heremans J. F. Lack of gamma A-immunoglobulin in serum of patients with steatorrhoea. Gut. 1966 Apr;7(2):119–127. doi: 10.1136/gut.7.2.119. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. EISEN A. H., KARPATI G., LASZLO T., ANDERMANN F., ROBB J. P., BACAL H. L. IMMUNOLOGIC DEFICIENCY IN ATAXIA TELANGIECTASIA. N Engl J Med. 1965 Jan 7;272:18–22. doi: 10.1056/NEJM196501072720104. [DOI] [PubMed] [Google Scholar]
  8. Ellis E. F., Smith R. T. The role of the spleen in immunity. With special reference to the post-splenectomy problem in infants. Pediatrics. 1966 Jan;37(1):111–119. [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., SOLOMON A. "Acquired agammaglobulinemia" with auto-immune hemolytic disease: graft-versus-host reaction? Vox Sang. 1961 Jan;6:68–79. doi: 10.1111/j.1423-0410.1961.tb03137.x. [DOI] [PubMed] [Google Scholar]
  11. GIEDION A., SCHEIDEGGER J. J. Kongenitale Immunparese bei Fehlen spezifischer beta 2-Globuline und quantitativ normalen gamma-Globulinen. Helv Paediatr Acta. 1957 Aug;12(3):241–259. [PubMed] [Google Scholar]
  12. GILBERT C., HONG R. QUALITATIVE AND QUANTITATIVE IMMUNOGLOBULIN DEFICIENCY. Am J Med. 1964 Oct;37:602–609. doi: 10.1016/0002-9343(64)90072-5. [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. HEREMANS J. F. Immunochemical studies on protein pathology. The immunoglobulin concept. Clin Chim Acta. 1959 Sep;4:639–646. doi: 10.1016/0009-8981(59)90004-x. [DOI] [PubMed] [Google Scholar]
  15. HINZ C. F., BOYER J. T. DYSGAMMAGLOBULINEMIA IN THE ADULT MANIFESTED AS AUTOIMMUNE HEMOLYTIC ANEMIA. SEROLOGIC AND IMMUNOCHEMICAL CHARACTERIZATION OF AN ANTIBODY OF UNUSUAL SPECIFICITY. N Engl J Med. 1963 Dec 19;269:1329–1335. doi: 10.1056/NEJM196312192692501. [DOI] [PubMed] [Google Scholar]
  16. HONG R., SCHUBERT W. K., PERRIN E. V., WEST C. D. Antibody deficiency syndrome associated with beta-2 macroglobulinemia. J Pediatr. 1962 Dec;61:831–842. doi: 10.1016/s0022-3476(62)80193-0. [DOI] [PubMed] [Google Scholar]
  17. HORAN M., COLEBATCH J. H. Relation between splenectomy and subsequent infection. A clinical study. Arch Dis Child. 1962 Aug;37:398–414. doi: 10.1136/adc.37.194.398. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. HUNTLEY C. C., LAFFERTY J. W., LYERLY A. ANTIBODY DEFICIENCY SYNDROME WITH INCREASED GAMMA1 M (B2 M) GLOBULINS AND ABSENT GAMMA1 A (B2 A) GLOBULINS AND GAMMA2 (G) GLOBULINS. Pediatrics. 1963 Sep;32:407–415. [PubMed] [Google Scholar]
  19. Hobbs J. R. Disturbances of the immunoglobulins. Sci Basis Med Annu Rev. 1966:106–127. [PubMed] [Google Scholar]
  20. ISRAEL-ASSELAIN R., BURTIN P., CHEBAT J. [A new biological disorder: agammaglobulinemia with beta2-macroglobulinemia (a case)]. Bull Mem Soc Med Hop Paris. 1960 Apr 1;76:519–523. [PubMed] [Google Scholar]
  21. KEKWICK R. A., VALLET L., CUTBUSH M., MOLLISON P. L., THOMAS A. R., GELL P. G., SOOTHILL J. F. Estimation of gamma globulin in the serum of patients with hypogammaglobulinaemia. J Clin Pathol. 1961 Sep;14:470–477. doi: 10.1136/jcp.14.5.470. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Lowdon A. G., Stewart R. H., Walker W. Risk of serious infection following splenectomy. Br Med J. 1966 Feb 19;1(5485):446–450. doi: 10.1136/bmj.1.5485.446. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. 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]
  24. Plotkin S. A., Klaus R. M., Whitely J. P. Hypogammaglobulinemia in an infant with congenital rubella syndrome; failure of 1-adamantanamine to stop irus excretion. J Pediatr. 1966 Dec;69(6):1085–1091. doi: 10.1016/s0022-3476(66)80299-8. [DOI] [PubMed] [Google Scholar]
  25. ROCKEY J. H., HANSON L. A., HEREMANS J. F., KUNKEL H. G. BETA-2A AGLOBULINEMIA IN TWO HEALTHY MEN. J Lab Clin Med. 1964 Feb;63:205–212. [PubMed] [Google Scholar]
  26. ROSEN F. S., BOUGAS J. A. ACQUIRED DYSGAMMAGLOBULINEMIA: ELEVATION OF THE 19S GAMMA GLOBULIN AND DEFICIENCY OF THE 7S GAMMA GLOBULIN IN A WOMAN WITH CHRONIC PROGRESSIVE BRONCHIECTASIS. N Engl J Med. 1963 Dec 19;269:1336–1340. doi: 10.1056/NEJM196312192692502. [DOI] [PubMed] [Google Scholar]
  27. ROSEN F. S., KEVY S. V., MERLER E., JANEWAY C. A., GITLIN D. Dysgammaglobulinaemia and recurrent bacterial infection. Lancet. 1961 Apr 1;1(7179):700–700. doi: 10.1016/s0140-6736(61)91725-1. [DOI] [PubMed] [Google Scholar]
  28. Soothill J. F., Hayes K., Dudgeon J. A. The immunoglobulins in congenital rubella. Lancet. 1966 Jun 25;1(7452):1385–1388. doi: 10.1016/s0140-6736(66)90299-6. [DOI] [PubMed] [Google Scholar]
  29. Stiehm E. R., Fudenberg H. H. Serum levels of immune globulins in health and disease: a survey. Pediatrics. 1966 May;37(5):715–727. [PubMed] [Google Scholar]
  30. THOMPSON E. N., JOHNSON R. S. A case of primary idiopathic hypogammaglobulinaemia associated with haemolytic anaemia. Postgrad Med J. 1962 May;38:292–295. doi: 10.1136/pgmj.38.439.292. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. WEST C. D., HONG R., HOLLAND N. H. Immunoglobulin levels from the newborn period to adulthood and in immunoglobulin deficiency states. J Clin Invest. 1962 Nov;41:2054–2064. doi: 10.1172/JCI104663. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. WOLF J. K., GOKCEN M., GOOD R. A. Heredo-familial disease of the mesenchymal tissues: clinical and laboratory study of one family. J Lab Clin Med. 1963 Feb;61:230–248. [PubMed] [Google Scholar]
  33. WOLLHEIM F. A., BELFRAGE S., COESTER C., LINDHOLM H. PRIMARY "ACQUIRED" HYPOGAMMAGLOBULINEMIA; CLINICAL AND GENETIC ASPECTS OF NINE CASES. Acta Med Scand. 1964 Jul;176:1–16. [PubMed] [Google Scholar]
  34. Williams R. T. Acquired dysgammaglobulinaemia in a young man. Clin Exp Immunol. 1966 Apr;1(2):223–231. [PMC free article] [PubMed] [Google Scholar]

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