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Immunoglobulin D Deficiency Clinical Presentation

  • Author: Donald A Dibbern, Jr, MD; Chief Editor: Michael A Kaliner, MD  more...
 
Updated: Mar 01, 2016
 

History

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  • 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

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  • 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

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  • 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, 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 College of Allergy, Asthma and Immunology, Clinical Immunology Society, Federation of American Societies for Experimental Biology, American Society for Virology, American Association of Immunologists, American Society for Microbiology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Michael R Simon, MD, MA Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Professor, Department of Internal Medicine, Oakland University William Beaumont 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, Michigan State Medical Society, Michigan Allergy and Asthma Society, American College of Physicians, American Federation for Medical Research, Royal College of Physicians and Surgeons of Canada, Society for Experimental Biology and Medicine

Disclosure: Received ownership interest from Secretory IgA, Inc. for management position; Received ownership interest from siRNAx, Inc. for management position.

Chief Editor

Michael A Kaliner, MD Clinical Professor of Medicine, George Washington University School of Medicine; 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, Association of American Physicians

Disclosure: Nothing to disclose.

Additional Contributors

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, Clinical Immunology Society

Disclosure: Received salary from CSL Behring for employment; Received ownership interest from CSL Behring for employment; Received consulting fee from America''s Health insurance plans for subject matter expert for clinical immunization safety assessment network acvtivity of cdc.

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

  2. Geisberger R, Lamers M, Achatz G. The riddle of the dual expression of IgM and IgD. Immunology. 2006 Aug. 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. 2009 Feb. 46(4):630-9. [Medline].

  5. Gambon-Deza F, Espinel CS. IgD in the reptile leopard gecko. Mol Immunol. 2008 Jul. 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. 2009 Aug. 10(8):889-98. [Medline]. [Full Text].

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

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

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

  10. Fraser PA, Schur PH. Hypoimmunoglobulinemia D: frequency, family studies, and association with HLA. Clin Immunol Immunopathol. 1981 Apr. 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. 2000 May. 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. 2000 Sep. 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. 2008 Aug 15. 198(4):481-5. [Medline].

  14. Sanal O, Ersoy F, Tezcan I, et al. Serum IgD concentrations in immunodeficiency diseases. Turk J Pediatr. 1990 Jul-Sep. 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. 1997 Sep. 114(1):54-8. [Medline].

  17. Haraldsson A, Weemaes CM, Jonasdottir S, et al. Serum immunoglobulin D in infants and children. Scand J Immunol. 2000 Apr. 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. 1980 Mar. 96(3 Pt 1):417-20. [Medline].

  19. Buckley RH, Fiscus SA. Serum IgD and IgE concentrations in immunodeficiency diseases. J Clin Invest. 1975 Jan. 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. 1992 Jun. 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. 2003 Jul. 23(4):297-305. [Medline].

  22. Lee SK, Metrakos JD, Tanaka KR, et al. Genetic influence on serum IgD levels. Pediatr Res. 1980 Jan. 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. 2000 Jun. 46(6 Pt 1):876-8. [Medline].

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

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

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