Abstract
Five patients with hyper-immunoglobulin D syndrome (hyper-IgD syndrome) were followed up for 3 to 8 years. In all patients studied, serum IgG3 was high. IgM decreased during the follow-up in all patients. In four of the patients serum IgA was elevated. In four patients the serum IgD κ/λ ratio was measured and was found to be raised in all. However, the serum total light-chain ratio and IgG, IgA, and IgM κ/λ ratios separately were virtually normal. In two of the patients, clinical symptoms preceded the increase in serum IgD. All patients had a history of severe reactions on immunizations in early childhood. We conclude that in hyper-IgD syndrome, other immunoglobulins may also be affected, in particular, IgA, IgM, and IgG3. The IgD light-chain ratio is also disturbed. We emphasize that clinical symptoms may herald immunological changes. This may be the result of an underlying factor causing both the clinical symptoms and, later, the increasing serum IgD levels.
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References
Hiemstra I, Vossn JM, van der Meer JWM, Weemaes CMR, Out TA, Zegers BJM: Clinical and immunological studies in patients with an increased serum IgD level. J Clin Immunol 9:393–400, 1989
Van der Meer JWM, Vossen JM, Radl J, van Nieuwenkoop JA, Meyer CJLM, Lobatto S, van Furth R: Hyperimmuno-globulinaemia D and periodic fever: a new syndrome. Lancet 1:1087–1090, 1984
Josephs SH, Buckley RH: Serum IgD concentrations in normal infants, children, and adults and in patients with elevated IgE. J Pediat 96:417–420, 1980
Blattner FR, Tucker PhW: The molecular biology of immunoglobulin D. Nature 307:417–422, 1984
Lafrenz D, Teale JM, Strober S: Role of IgD in immunological memory. Ann NY Acad Sci 399:375–388, 1982
Coico R, Xue B, Wallace D, Pernis B, Siskind GW, Thorbecke GJ: T cells with receptor for IgD. Nature 316:744–746, 1985
Haraldsson Á, Jaminon M, Bakkeren JAJM, Stoelinga GBA, Weemaes CMR: Immunoglobulin G, A, and M light chain ratio in some humoral immunological disorders. Scand J Immunol 36:57–61, 1992
Renckens ALJM, Jansen MJH, Van Munster PJJ, Weemaes CMR, Bakkeren JAJM: Nephelometry of the kappa/lambda light chain ratio in serum of normal and diseased children. Clin Chem 32:2147–2149, 1986
Jasani B: Immunohistologically definable light chain restriction in autoimmuno disease. J Pathol 154:1–5, 1988
Knight J, Laing P, Knight A, Adams D, Ling N: Thyroid-stimulation autoantibodies usually contain only L-light chains: Evidence for the “forbidden clone” theory. J Clin Endocrinol Metab 62:342–347, 1986
Carson DA, Lawrance S: Light chain heterogeneity of 19S and 7S anti-g-globulins in rheumatoid arthritis and subacute bacterial endocarditis. Arth Rheum 21:438–440, 1978
Jonsson T, Arnason JA, Valdimarsson H: Enzyme-linked immunosorbent assay (ELISA) screening test for detection of rheumatoid factor. Rheumatology 6:199–204, 1986
Haraldsson Á, Kock-Jansen MJH, Jaminon M, van Eck-Arts PBJM, de Boo T, Weemaes CMR, Bakkeren JAJM: Determination of kappa and lambda light chains in serum immunoglobulins G, A, and M. Ann Clin Biochem 28:461–466, 1991
Van Munster PJJ, Stoelinga GBA: Over de isolering en bepaling van immunoglobulin A (IgA). Maandschr Kindergeneesk 33:428–433, 1965
Plebani A, Ugazio AG, Avanzini MA, Massimi P, Zonta L, Monafo V, Burgio GR. Serum IgG subclass concentrations in healthy subjects at different age: Age normal percentile charts. Eur J Pediat 149:164–167, 1989
Haraldsson Á, Weemaes CMR, Kock-Jansen MJH, van Eck-Arts PBJM, de Boo T, Bakkeren JAJM, Stoelinga GBA: Immunoglobulin G, A, and M light chain ratio in children. Ann Clin Biochem 29:270–274, 1992
Van Nieuwenkoop JA, Radl J: Light chain types of IgD in human bone marrow and serum. Clin Exp Immunol 60:654–660, 1985
Corte G, Torda P, Cosulich E, Milstein CP, Bargellesi A, Ferrarini M: Characterization of IgD. I. Isolation of two molecular forms from human serum. Scand J Immunol 9:141–149, 1979
Vossen JM: The Development of the B Immune System in Man, Thesis. Leiden, The Netherlands, 1975
Weemaes CMR, Bakkeren JAJM, ter Haar BGA, Hustinx TWJ, van Munster PJJ: Immune response in four patients with Bloom syndrome. Clin Immunol Immunopath 12:12–19, 1975
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Haraldsson, Á., Weemaes, C.M.R., de Boer, A.W. et al. Immunological studies in the hyper-immunoglobulin D syndrome. J Clin Immunol 12, 424–428 (1992). https://doi.org/10.1007/BF00918854
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DOI: https://doi.org/10.1007/BF00918854