Immunoglobulin D Deficiency Clinical Presentation

  • Author: Donald A Dibbern Jr, MD; Chief Editor: Michael A Kaliner, MD   more...
 
Updated: Jul 22, 2011
 

History

  • No specific signs or symptoms are associated with isolated IgD deficiency; therefore, this condition is usually discovered incidentally during immunological laboratory testing (eg, quantitative serum immunoglobulin levels).
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Physical

  • A patient with low IgD levels but no concurrent immunoglobulin deficiencies of other classes or other immune defects typically does not develop specific physical findings associated with low or absent IgD levels.
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Causes

  • Family studies from one report indicate that low serum IgD levels may be inherited in an autosomal recessive fashion.[9]
  • Another study found several families with possible characteristics of autosomal recessive inheritance, and other families with a pattern more consistent with multiple allele involvement. This latter report also suggested an increased frequency of certain HLA antigens in individuals with low IgD levels.[10]
  • An HLA association has also been seen in a Basque population, which suggested a partially penetrant dominant susceptibility gene for IgD deficiency.[11] These findings have been further supported in another recent study.[12]
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Contributor Information and Disclosures
Author

Donald A Dibbern Jr, MD  Consulting Staff (Allergist), Providence St Vincent Medical Center

Donald A Dibbern Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, and Oregon Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

John M Routes, MD  Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics, Chief, Section of Allergy and Clinical Immunology, Department of Pediatrics, Medical College of Wisconsin

John M Routes, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Microbiology, American Society for Virology, Clinical Immunology Society, and Federation of American Societies for Experimental Biology

Disclosure: Nothing to disclose.

Specialty Editor Board

Melvin Berger, MD, PhD  Adjunct Professor of Pediatrics and Pathology, Case Western Reserve University; Senior Medical Director, Clinical Research and Development, CSL Behring, LLC

Melvin Berger, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Clinical Investigation, and Clinical Immunology Society

Disclosure: CSL Behring Salary Employment; CSL Behring Ownership interest Employment; America's Health insurance plans Consulting fee Subject Matter Expert For Clinical Immunization Safety Assessment Network acvtivity of CDC

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Michael R Simon, MD, MA  Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital

Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, and Society for Experimental Biology and Medicine

Disclosure: Secretory IgA, Inc. Ownership interest Management position

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Michael A Kaliner, MD  Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy

Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians

Disclosure: Alcon Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering/Merck Consulting fee Consulting; Teva Consulting fee Consulting; Meda Honoraria Speaking and teaching; Ista Consulting

References

  1. Levan-Petit I, Lelievre E, Barra A, et al. Th2 cytokine dependence of IgD production by normal human B cells. Int Immunol. 1999;11:1819-1828.

  2. Geisberger R, Lamers M, Achatz G. The riddle of the dual expression of IgM and IgD. Immunology. Aug 2006;118(4):429-37. [Medline].

  3. Lutz C, Ledermann B, Kosco-Vilbois MH, et al. IgD can largely substitute for loss of IgM function in B cells. Nature. 1998;393 (6687):797-801.

  4. Seifert M, Steimle-Grauer SA, Goossens T, Hansmann ML, Brauninger A, Kuppers R. A model for the development of human IgD-only B cells: Genotypic analyses suggest their generation in superantigen driven immune responses. Mol Immunol. Feb 2009;46(4):630-9. [Medline].

  5. Gambon-Deza F, Espinel CS. IgD in the reptile leopard gecko. Mol Immunol. Jul 2008;45(12):3470-6. [Medline].

  6. Chen K, Xu W, Wilson M, et al. Immunoglobulin D enhances immune surveillance by activating antimicrobial, proinflammatory and B cell-stimulating programs in basophils. Nat Immunol. Aug 2009;10(8):889-98. [Medline]. [Full Text].

  7. Chen K, Cerutti A. New insights into the enigma of immunoglobulin D. Immunol Rev. Sep 2010;237(1):160-79. [Medline]. [Full Text].

  8. Chen K, Cerutti A. The function and regulation of immunoglobulin D. Curr Opin Immunol. Jun 2011;23(3):345-52. [Medline]. [Full Text].

  9. Dunnette SL, Gleich GJ, Weinshilboum RM. Inheritance of low serum immunoglobulin D. J Clin Invest. Aug 1978;62(2):248-55. [Medline].

  10. Fraser PA, Schur PH. Hypoimmunoglobulinemia D: frequency, family studies, and association with HLA. Clin Immunol Immunopathol. Apr 1981;19(1):67-74. [Medline].

  11. Calvo B, Castano L, Marcus-Bagley D, et al. The [HLA-B18, F1C30, DR3] conserved extended haplotype carries a susceptibility gene for IgD deficiency. J Clin Immunol. May 2000;20(3):216-20. [Medline].

  12. Alper CA, Marcus-Bagley D, Awdeh Z, et al. Prospective analysis suggests susceptibility genes for deficiencies of IgA and several other immunoglobulins on the [HLA-B8, SC01, DR3] conserved extended haplotype. Tissue Antigens. Sep 2000;56(3):207-16. [Medline].

  13. Jacobsen MC, Thiebaut R, Fisher C, Sefe D, Clapson M, Klein N. Pediatric human immunodeficiency virus infection and circulating IgD+ memory B cells. J Infect Dis. Aug 15 2008;198(4):481-5. [Medline].

  14. Sanal O, Ersoy F, Tezcan I, et al. Serum IgD concentrations in immunodeficiency diseases. Turk J Pediatr. Jul-Sep 1990;32(3):175-82. [Medline].

  15. Mosedale DE, Sandhu MS, Luan J, Goodall M, Grainger DJ. A new sensitive and specific enzyme-linked immunosorbent assay for IgD. J Immunol Methods. 2006;313(1-2):74-80. [Medline].

  16. Litzman J, Ward AM, Wild G, et al. Serum IgD levels in children under investigation for and with defined immunodeficiency. Int Arch Allergy Immunol. Sep 1997;114(1):54-8. [Medline].

  17. Haraldsson A, Weemaes CM, Jonasdottir S, et al. Serum immunoglobulin D in infants and children. Scand J Immunol. Apr 2000;51(4):415-8. [Medline].

  18. Josephs SH, Buckley RH. Serum IgD concentrations in normal infants, children, and adults and in patients with elevated IgE. J Pediatr. Mar 1980;96(3 Pt 1):417-20. [Medline].

  19. Buckley RH, Fiscus SA. Serum IgD and IgE concentrations in immunodeficiency diseases. J Clin Invest. Jan 1975;55(1):157-65. [Medline].

  20. de Laat PC, Weemaes CM, Bakkeren JA. Immunoglobulin levels during follow-up of children with selective IgA deficiency. Scand J Immunol. Jun 1992;35(6):719-25. [Medline].

  21. Alper CA, Xu J, Cosmopoulos K, et al. Immunoglobulin deficiencies and susceptibility to infection among homozygotes and heterozygotes for C2 deficiency. J Clin Immunol. Jul 2003;23(4):297-305. [Medline].

  22. Lee SK, Metrakos JD, Tanaka KR, et al. Genetic influence on serum IgD levels. Pediatr Res. Jan 1980;14(1):60-3. [Medline].

  23. Levan-Petit I, Cardonna J, Garcia M, et al. Sensitive ELISA for human immunoglobulin D measurement in neonate, infant, and adult sera. Clin Chem. Jun 2000;46(6 Pt 1):876-8. [Medline].

  24. Vladutiu AO. Immunoglobulin D: properties, measurement, and clinical relevance. Clin Diagn Lab Immunol. Mar 2000;7(2):131-40. [Medline].

  25. Vladutiu AO, Netto D. Is quantitation of serum IgD clinically useful? [letter]. Clin Chem. Jun 1982;28(6):1409-10. [Medline].

 
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