Immunoglobulin D Deficiency
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Immunoglobulin D (IgD) deficiency is a defect of humoral immunity that is characterized by abnormally low serum levels of IgD immunoglobulins. Little is known about the normal function of IgD, and few clinical signs or symptoms are associated with its absence. Individuals with low or absent levels of IgD do not appear unusually predisposed to infections.
Genetic rearrangements occur during the maturation of B lymphocytes, eventually resulting in the surface expression of both immunoglobulin M (IgM) and IgD on mature B cells. Cell signaling occurs through this surface IgD. IgD production by B cells is stimulated by interleukin (IL)–4 and IL-10. [1]
The physiologic purpose of free serum IgD is not well understood, though it may fine tune or modulate humoral immune response. [2] In mice, IgD may substitute for some functions of IgM when IgM is absent. Studies in IgM-deficient IgM-/- mice reveal that B cells with surface expression of IgM were replaced by B cells with surface expression of IgD. Immunization of IgM-/- mice revealed an IgD immune response in place of the now absent IgM response, although with a delayed increase in antibody concentration as compared to normal. [3] Recent studies have suggested that IgD-only B cells may play a significant role in immune responses to superantigens. [4] Investigations into the evolutionary origins of IgD are also ongoing. [5, 6, 7, 8]
Low serum IgD levels are not distributed in a normal gaussian fashion. [9, 10] IgD deficiency is associated with the specific human leukocyte antigens HLA-B18, F1C30, and DR3 in a Spanish Basque population [11] and HLA-B8, SC01, DR3 in white subjects in an American study. [12] A 2008 report noted that depletion of circulating IgD+ memory B cells occurs in pediatric HIV infection, despite control of viral load with highly active anti-retroviral therapy (HAART). [13]
United States
One report indicates that approximately 11% of 371 American Red Cross blood donors and 6% of 1529 study subjects had low or undetectable IgD levels (< 0.002 mg/mL). In the study group, a number of the individuals with low IgD had rheumatologic disease (eg, juvenile rheumatoid arthritis, lupus, psoriatic arthritis, vasculitis), but the frequency of low IgD within groups of patients with each disease did not differ from the normal controls using chi-square analysis. [10] In another study, using a cutoff of 2.15 IU/mL, assays of 245 healthy adults and 301 healthy children revealed that approximately 13% of each group had low levels of IgD. [9]
Low or undetectable levels of IgD, in the absence of other concurrent disease or immune defects (eg, common variable immunodeficiency, complement deficiency), are not associated with morbidity or increased mortality. Specifically, patients with low or undetectable IgD levels do not demonstrate an increased incidence of infections of any type. [14]
Overall levels of serum IgD are higher in males than females, [15] but specific incidence of abnormally low IgD is approximately equal between the sexes. [9]
Children younger than 3 years, both with and without an immunodeficiency, appear to have an increased prevalence of low IgD levels. [16, 17, 18] After infancy, age is not associated with increased prevalence of low IgD levels. [9]
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.
Geisberger R, Lamers M, Achatz G. The riddle of the dual expression of IgM and IgD. Immunology. 2006 Aug. 118(4):429-37. [Medline].
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.
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].
Gambon-Deza F, Espinel CS. IgD in the reptile leopard gecko. Mol Immunol. 2008 Jul. 45(12):3470-6. [Medline].
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].
Chen K, Cerutti A. New insights into the enigma of immunoglobulin D. Immunol Rev. 2010 Sep. 237(1):160-79. [Medline]. [Full Text].
Chen K, Cerutti A. The function and regulation of immunoglobulin D. Curr Opin Immunol. 2011 Jun. 23(3):345-52. [Medline]. [Full Text].
Dunnette SL, Gleich GJ, Weinshilboum RM. Inheritance of low serum immunoglobulin D. J Clin Invest. 1978 Aug. 62(2):248-55. [Medline].
Fraser PA, Schur PH. Hypoimmunoglobulinemia D: frequency, family studies, and association with HLA. Clin Immunol Immunopathol. 1981 Apr. 19(1):67-74. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
Buckley RH, Fiscus SA. Serum IgD and IgE concentrations in immunodeficiency diseases. J Clin Invest. 1975 Jan. 55(1):157-65. [Medline].
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].
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].
Lee SK, Metrakos JD, Tanaka KR, et al. Genetic influence on serum IgD levels. Pediatr Res. 1980 Jan. 14(1):60-3. [Medline].
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].
Vladutiu AO. Immunoglobulin D: properties, measurement, and clinical relevance. Clin Diagn Lab Immunol. 2000 Mar. 7(2):131-40. [Medline].
Vladutiu AO, Netto D. Is quantitation of serum IgD clinically useful? [letter]. Clin Chem. 1982 Jun. 28(6):1409-10. [Medline].
Camellia L Hernandez, MD Fellow in Allergy/Immunology, Walter Reed National Military Medical Center
Camellia L Hernandez, MD 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 Chest Physicians, American College of Physicians, Massachusetts Medical Society
Disclosure: Nothing to disclose.
Taylor Banks, MD Chief, Allergy/Immunology Clinic, Walter Reed National Military Medical Center; Assistant Professor of Pediatrics, Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Associate Program Director, NCC Allergy-Immunology Fellowship Program
Taylor Banks, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.
Jeannie Lee Bay, DO Fellow in Allergy/Immunology, Walter Reed National Military Medical Center; Assistant Professor of Pediatrics, Instructor of Medicine, Department of Pediatrics, Uniformed Services University of the Health Sciences
Jeannie Lee Bay, DO is a member of the following medical societies: American Academy of Allergy, Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, American Osteopathic Association, Association of Military Osteopathic Physicians and Surgeons
Disclosure: Nothing to disclose.
Evelyn M Lomasney, MD Fellow in Allergy/Immunology, Walter Reed National Military Medical Center; Assistant Professor, Department of Medicine, Uniformed Services University of the Health Sciences
Evelyn M Lomasney, MD 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
Disclosure: Nothing to disclose.
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, 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, Society for Experimental Biology and Medicine
Disclosure: Have a 5% or greater equity interest in: Secretory IgA, Inc. ; siRNAx, Inc.<br/>Received income in an amount equal to or greater than $250 from: siRNAx, Inc.
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.
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.
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.
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.
Immunoglobulin D Deficiency
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