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Selective immunoglobulin deficiency disease

MedGen UID:
235584
•Concept ID:
C1335942
•
Disease or Syndrome
Synonyms: selective immunoglobulin deficiency disease; Selective Immunoglobulin Isotype Deficiency; selective Immunoglobulin isotype deficiency
 
Monarch Initiative: MONDO:0003739

Definition

A broad classification of dysgammaglobulinemias characterized by low or undetectable serum levels of one of the five immunoglobulin classes. Deficiencies of immunoglobulins present variably according to isotype. Selective deficiencies may be caused by decreased or inefficient production from progenitor B cells without any corresponding decreases in the other isotypes. The clinical course and prognosis is dependent upon the severity of the selective deficiency and associated morbidity. [from NCI]

Professional guidelines

PubMed

Antibiotic Prophylaxis for Dental Treatment in Patients with Immunodeficiency.
Squire JD, Gardner PJ, Moutsopoulos NM, Leiding JW
J Allergy Clin Immunol Pract 2019 Mar;7(3):819-823. Epub 2019 Jan 21 doi: 10.1016/j.jaip.2019.01.016. PMID: 30677537
Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.
Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681
Management of VWD.
Neff AT, Sidonio RF Jr
Hematology Am Soc Hematol Educ Program 2014 Dec 5;2014(1):536-41. Epub 2014 Nov 18 doi: 10.1182/asheducation-2014.1.536. PMID: 25696907

Recent clinical studies

Etiology

Loss of tolerance precedes triggering and lifelong persistence of pathogenic type I interferon autoantibodies.
Fernbach S, Mair NK, Abela IA, Groen K, Kuratli R, Lork M, Thorball CW, Bernasconi E, Filippidis P, Leuzinger K, Notter J, Rauch A, Hirsch HH, Huber M, Günthard HF, Fellay J, Kouyos RD, Hale BG; Swiss HIV Cohort Study
J Exp Med 2024 Sep 2;221(9) Epub 2024 Jul 17 doi: 10.1084/jem.20240365. PMID: 39017930Free PMC Article
Prevalence of neurological diseases among patients with selective IgA deficiency.
Magen E, Geishin A, Merzon E, Green I, Golan-Cohen A, Vinker S, Israel A
Allergy Asthma Proc 2023 Sep 1;44(5):e17-e21. doi: 10.2500/aap.2023.44.230036. PMID: 37641224
Update on Infections in Primary Antibody Deficiencies.
Demirdag YY, Gupta S
Front Immunol 2021;12:634181. Epub 2021 Feb 11 doi: 10.3389/fimmu.2021.634181. PMID: 33643318Free PMC Article
Personalized Therapy: Immunoglobulin Replacement for Antibody Deficiency.
Wasserman RL
Immunol Allergy Clin North Am 2019 Feb;39(1):95-111. doi: 10.1016/j.iac.2018.08.001. PMID: 30466775
Ataxia-telangiectasia: Immunodeficiency and survival.
van Os NJH, Jansen AFM, van Deuren M, Haraldsson A, van Driel NTM, Etzioni A, van der Flier M, Haaxma CA, Morio T, Rawat A, Schoenaker MHD, Soresina A, Taylor AMR, van de Warrenburg BPC, Weemaes CMR, Roeleveld N, Willemsen MAAP
Clin Immunol 2017 May;178:45-55. Epub 2017 Jan 24 doi: 10.1016/j.clim.2017.01.009. PMID: 28126470

Diagnosis

Update on Infections in Primary Antibody Deficiencies.
Demirdag YY, Gupta S
Front Immunol 2021;12:634181. Epub 2021 Feb 11 doi: 10.3389/fimmu.2021.634181. PMID: 33643318Free PMC Article
B Cell Disorders in Children: Part II.
Griffin DD, Dolen WK
Curr Allergy Asthma Rep 2020 Aug 21;20(11):64. doi: 10.1007/s11882-020-00963-z. PMID: 32821980
The Treatment of Activated PI3Kδ Syndrome.
Coulter TI, Cant AJ
Front Immunol 2018;9:2043. Epub 2018 Sep 7 doi: 10.3389/fimmu.2018.02043. PMID: 30245694Free PMC Article
Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.
Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681
Ataxia telangiectasia: a review.
Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM
Orphanet J Rare Dis 2016 Nov 25;11(1):159. doi: 10.1186/s13023-016-0543-7. PMID: 27884168Free PMC Article

Therapy

Antibody deficiency testing for primary immunodeficiency: A practical review for the clinician.
Marsh RA, Orange JS
Ann Allergy Asthma Immunol 2019 Nov;123(5):444-453. Epub 2019 Aug 23 doi: 10.1016/j.anai.2019.08.012. PMID: 31446132
Targeting Interleukin-6 Signaling in Clinic.
Kang S, Tanaka T, Narazaki M, Kishimoto T
Immunity 2019 Apr 16;50(4):1007-1023. doi: 10.1016/j.immuni.2019.03.026. PMID: 30995492
The Treatment of Activated PI3Kδ Syndrome.
Coulter TI, Cant AJ
Front Immunol 2018;9:2043. Epub 2018 Sep 7 doi: 10.3389/fimmu.2018.02043. PMID: 30245694Free PMC Article
Stromal elements act to restrain, rather than support, pancreatic ductal adenocarcinoma.
Rhim AD, Oberstein PE, Thomas DH, Mirek ET, Palermo CF, Sastra SA, Dekleva EN, Saunders T, Becerra CP, Tattersall IW, Westphalen CB, Kitajewski J, Fernandez-Barrena MG, Fernandez-Zapico ME, Iacobuzio-Donahue C, Olive KP, Stanger BZ
Cancer Cell 2014 Jun 16;25(6):735-47. Epub 2014 May 22 doi: 10.1016/j.ccr.2014.04.021. PMID: 24856585Free PMC Article
Mechanisms of action of intravenous immunoglobulin in autoimmune and inflammatory diseases.
Bayry J, Misra N, Latry V, Prost F, Delignat S, Lacroix-Desmazes S, Kazatchkine MD, Kaveri SV
Transfus Clin Biol 2003 Jun;10(3):165-9. doi: 10.1016/s1246-7820(03)00035-1. PMID: 12798851

Prognosis

Update on Infections in Primary Antibody Deficiencies.
Demirdag YY, Gupta S
Front Immunol 2021;12:634181. Epub 2021 Feb 11 doi: 10.3389/fimmu.2021.634181. PMID: 33643318Free PMC Article
Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.
Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681
Ataxia telangiectasia: a review.
Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM
Orphanet J Rare Dis 2016 Nov 25;11(1):159. doi: 10.1186/s13023-016-0543-7. PMID: 27884168Free PMC Article
Stromal elements act to restrain, rather than support, pancreatic ductal adenocarcinoma.
Rhim AD, Oberstein PE, Thomas DH, Mirek ET, Palermo CF, Sastra SA, Dekleva EN, Saunders T, Becerra CP, Tattersall IW, Westphalen CB, Kitajewski J, Fernandez-Barrena MG, Fernandez-Zapico ME, Iacobuzio-Donahue C, Olive KP, Stanger BZ
Cancer Cell 2014 Jun 16;25(6):735-47. Epub 2014 May 22 doi: 10.1016/j.ccr.2014.04.021. PMID: 24856585Free PMC Article
Selective IgA deficiency.
Burks AW Jr, Steele RW
Ann Allergy 1986 Jul;57(1):3-13. PMID: 3487995

Clinical prediction guides

Prevalence of neurological diseases among patients with selective IgA deficiency.
Magen E, Geishin A, Merzon E, Green I, Golan-Cohen A, Vinker S, Israel A
Allergy Asthma Proc 2023 Sep 1;44(5):e17-e21. doi: 10.2500/aap.2023.44.230036. PMID: 37641224
Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond.
Favaloro EJ, Pasalic L, Henry B, Lippi G
Am J Hematol 2021 Aug 1;96(8):1049-1055. Epub 2021 May 31 doi: 10.1002/ajh.26241. PMID: 33991361
Stromal elements act to restrain, rather than support, pancreatic ductal adenocarcinoma.
Rhim AD, Oberstein PE, Thomas DH, Mirek ET, Palermo CF, Sastra SA, Dekleva EN, Saunders T, Becerra CP, Tattersall IW, Westphalen CB, Kitajewski J, Fernandez-Barrena MG, Fernandez-Zapico ME, Iacobuzio-Donahue C, Olive KP, Stanger BZ
Cancer Cell 2014 Jun 16;25(6):735-47. Epub 2014 May 22 doi: 10.1016/j.ccr.2014.04.021. PMID: 24856585Free PMC Article
Current relevance of pharmacogenetics in immunomodulation treatment for Crohn's disease.
Roberts RL, Barclay ML
J Gastroenterol Hepatol 2012 Oct;27(10):1546-54. doi: 10.1111/j.1440-1746.2012.07220.x. PMID: 22741564
Mechanisms of immunosuppression.
Dohms JE
Vet Immunol Immunopathol 1991 Nov;30(1):19-30. doi: 10.1016/0165-2427(91)90005-w. PMID: 1781154

Recent systematic reviews

Meta-analysis and systematic review: burosumab as a promising treatment for children with X-linked hypophosphatemia.
Wang K, Zhang R, Chen Z, Bai Y, He Q
Front Endocrinol (Lausanne) 2024;15:1414509. Epub 2024 Aug 15 doi: 10.3389/fendo.2024.1414509. PMID: 39211452Free PMC Article
Prevalence of Toxoplasma gondii in HIV-Infected Individuals in Iran: A Systematic Review and Meta-Analysis.
Shakib P, Behzadifar M, Mahmoudvand H, Larki S, Kalani H, Zivdari M, Mardanshah O, Cheraghipour K
Curr HIV Res 2023;21(4):221-231. doi: 10.2174/011570162X244384230920033134. PMID: 37873950
Isolated ACTH deficiency induced by cancer immunotherapy: a systematic review.
Iglesias P, Sánchez JC, Díez JJ
Pituitary 2021 Aug;24(4):630-643. Epub 2021 Mar 24 doi: 10.1007/s11102-021-01141-8. PMID: 33761049
Enzyme replacement therapy with laronidase (Aldurazyme(®)) for treating mucopolysaccharidosis type I.
Jameson E, Jones S, Remmington T
Cochrane Database Syst Rev 2016 Apr 1;4:CD009354. doi: 10.1002/14651858.CD009354.pub4. PMID: 27033167
Clinical inquiries: What blood tests help diagnose celiac disease?
Reddick BK, Crowell K, Fu B
J Fam Pract 2006 Dec;55(12):1088, 1090, 1093. PMID: 17137549

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