Hypogammaglobulinemia

References

Key articles

Tangye SG, Al-Herz W, Bousfiha A, et al. Human inborn errors of immunity: 2022 update on the classification from the International Union of Immunological Societies Expert Committee J Clin Immunol. 2022 Jun 24;1-35 [Epub ahead of print].Full text  Abstract

Otani IM, Lehman HK, Jongco AM, et al. Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: a Work Group report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees. J Allergy Clin Immunol. 2022 May;149(5):1525-60.Full text  Abstract

Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136(5):1186-205;e1-78.Full text  Abstract

Kohn DB, Hershfield MS, Puck JM, et al. Consensus approach for the management of severe combined immune deficiency caused by adenosine deaminase deficiency. J Allergy Clin Immunol. 2019 Mar;143(3):852-63.Full text  Abstract

National Blood Authority, Australia. Criteria for the clinical use of immunoglobulin in Australia: version 3. 2018 [internet publication].Full text

Reference articles

1. Tangye SG, Al-Herz W, Bousfiha A, et al. Human inborn errors of immunity: 2022 update on the classification from the International Union of Immunological Societies Expert Committee J Clin Immunol. 2022 Jun 24;1-35 [Epub ahead of print].Full text  Abstract

2. Otani IM, Lehman HK, Jongco AM, et al. Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: a Work Group report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees. J Allergy Clin Immunol. 2022 May;149(5):1525-60.Full text  Abstract

3. Onigbanjo M, Orange J, Perez E, et al. Hypogammaglobulinemia in a pediatric tertiary care setting. Clin Immunol. 2007 Oct;125(1):52-9. Abstract

4. Kobrynski L, Powell RW, Bowen S. Prevalence and morbidity of primary immunodeficiency diseases, United States 2001-2007. J Clin Immunol. 2014 Nov;34(8):954-61.Full text  Abstract

5. Rubin Z, Pappalardo A, Schwartz A, et al. Prevalence and outcomes of primary immunodeficiency in hospitalized children in the United States. J Allergy Clin Immunol Pract. 2018 Sep-Oct;6(5):1705-10;e1. Abstract

6. Kirkpatrick P, Riminton S. Primary immunodeficiency diseases in Australia and New Zealand. J Clin Immunol. 2007 Sep;27(5):517-24. Abstract

7. Slade CA, Bosco JJ, Binh Giang T, et al. Delayed diagnosis and complications of predominantly antibody deficiencies in a cohort of Australian adults. Front Immunol. 2018 May 14;9:694.Full text  Abstract

8. Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136(5):1186-205;e1-78.Full text  Abstract

9. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999 Dec;93(3):190-7. Abstract

10. Kwan A, Abraham RS, Currier R, et al. Newborn screening for severe combined immunodeficiency in 11 screening programs in the United States. JAMA. 2014 Aug 20;312(7):729-38.Full text  Abstract

11. Quinti I, Soresina A, Spadaro G, et al; Italian Primary Immunodeficiency Network. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007 May;27(3):308-16. Abstract

12. Wood P, Stanworth S, Burton J, et al. Recognition, clinical diagnosis and management of patients with primary antibody deficiencies: a systematic review. Clin Exp Immunol. 2007 Sep;149(3):410-23.Full text  Abstract

13. Bonilla FA, Barlan I, Chapel H, et al. International consensus document (ICON): common variable immunodeficiency disorders. J Allergy Clin Immunol Pract. 2016 Jan-Feb;4(1):38-59. Abstract

14. Bonilla FA, Geha RS. Common variable immunodeficiency. Pediatr Res. 2009 May;65(5 pt 2):13-19R.Full text  Abstract

15. Bogaert DJ, Dullaers M, Lambrecht BN, et al. Genes associated with common variable immunodeficiency: one diagnosis to rule them all? J Med Genet. 2016 Sep;53(9):575-90.Full text  Abstract

16. Patel SY, Carbone J, Jolles S. The expanding field of secondary antibody deficiency: causes, diagnosis, and management. Front Immunol. 2019 Feb 8;10:33.Full text  Abstract

17. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 2007 Sep;149(3):410-23. Abstract

18. Duan L, Grunebaum E. Hematological malignancies associated with primary immunodeficiency disorders. Clin Immunol. 2018 Sep;194:46-59. Abstract

19. Riaz IB, Faridi W, Patnaik MM, et al. A systematic review on predisposition to lymphoid (B and T cell) neoplasias in patients with primary immunodeficiencies and immune dysregulatory disorders (inborn errors of immunity). Front Immunol. 2019 Apr 16;10:777.Full text  Abstract

20. Levy R, Mahévas M, Galicier L, et al. Profound symptomatic hypogammaglobulinemia: a rare late complication after rituximab treatment for immune thrombocytopenia. Report of 3 cases and systematic review of the literature. Autoimmun Rev. 2014 Oct;13(10):1055-63. Abstract

21. Christou EA, Giardino G, Worth A, et al. Risk factors predisposing to the development of hypogammaglobulinemia and infections post-rituximab. Int Rev Immunol. 2017 Nov 2;36(6):352-9. Abstract

22. Khan DA. Hypersensitivity and immunologic reactions to biologics: opportunities for the allergist. Ann Allergy Asthma Immunol. 2016 Aug;117(2):115-20.Full text  Abstract

23. Ottaviano G, Marinoni M, Graziani S, et al. Rituximab unveils hypogammaglobulinemia and immunodeficiency in children with autoimmune cytopenia. J Allergy Clin Immunol Pract. 2020 Jan;8(1):273-82. Abstract

24. Kilic SS, Tezcan I, Sanal O, et al. Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases. Pediatr Int. 2000 Dec;42(6):647-50. Abstract

25. Kohn DB, Hershfield MS, Puck JM, et al. Consensus approach for the management of severe combined immune deficiency caused by adenosine deaminase deficiency. J Allergy Clin Immunol. 2019 Mar;143(3):852-63.Full text  Abstract

26. Mazza JM, Lin SY. Primary immunodeficiency and recalcitrant chronic sinusitis: a systematic review. Int Forum Allergy Rhinol. 2016 Oct;6(10):1029-33. Abstract

27. Schwitzguébel AJ, Jandus P, Lacroix JS, et al. Immunoglobulin deficiency in patients with chronic rhinosinusitis: systematic review of the literature and meta-analysis. J Allergy Clin Immunol. 2015 Dec;136(6):1523-31.Full text  Abstract

28. Dong JP, Gao W, Teng GG, et al. Characteristics of Good's syndrome in China: a systematic review. Chin Med J (Engl). 2017 Jul 5;130(13):1604-9.Full text  Abstract

29. Touw CM, van de Ven AA, de Jong PA, et al. Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allerg Immunol. 2010;21:793-805. Abstract

30. Oliveira JB, Notarangelo LD, Fleisher TA. Applications of flow cytometry for the study of primary immune deficiencies. Curr Opin Allergy Clin Immunol. 2008 Dec;8(6):499-509. Abstract

31. Centers for Disease Control and Prevention. Severe Combined Immunodeficiency (SCID). Mar 2019 [internet publication].Full text

32. van der Burg M, Mahlaoui N, Gaspar HB, et al. Universal Newborn Screening for Severe Combined Immunodeficiency (SCID). Front Pediatr. 2019;7:373.Full text  Abstract

33. de Vries E; European Society for Immunodeficiencies (ESID) members. Patient-centred screening for primary immunodeficiency, a multi-stage diagnostic protocol designed for non-immunologists: 2011 update. Clin Exp Immunol. 2012 Jan;167(1):108-19.Full text  Abstract

34. Gardulf A, Andersen V, Bjorkander J, et al. Subcutaneous immunoglobulin replacement in patients with primary antibody deficiencies: safety and costs. Lancet. 1995 Feb 11;345(8946):365-9. Abstract

35. Chapel HM, Spickett GP, Ericson D, et al. The comparison of the efficacy and safety of intravenous versus subcutaneous immunoglobulin replacement therapy. J Clin Immunol. 2000 Mar;20(2):94-100. Abstract

36. Na IK, Buckland M, Agostini C, et al. Current clinical practice and challenges in the management of secondary immunodeficiency in hematological malignancies. Eur J Haematol. 2019 Jun;102(6):447-56.Full text  Abstract

37. Shrestha P, Karmacharya P, Wang Z, et al. Impact of IVIG vs. SCIG on IgG trough level and infection incidence in primary immunodeficiency diseases: A systematic review and meta-analysis of clinical studies. World Allergy Organ J. 2019 Oct;12(10):100068.Full text  Abstract

38. Shabaninejad H, Asgharzadeh A, Rezaei N, et al. A comparative study of intravenous immunoglobulin and subcutaneous immunoglobulin in adult patients with primary immunodeficiency diseases: a systematic review and meta-analysis. Expert Rev Clin Immunol. 2016;12(5):595-602. Abstract

39. Horn J, Thon V, Bartonkova D, et al. Anti-IgA antibodies in common variable immunodeficiency (CVID): diagnostic workup and therapeutic strategy. Clin Immunol. 2007 Feb;122(2):156-62. Abstract

40. Abolhassani H, Sadaghiani MS, Aghamohammadi A, et al. Home-based subcutaneous immunoglobulin versus hospital-based intravenous immunoglobulin in treatment of primary antibody deficiencies: systematic review and meta analysis. J Clin Immunol. 2012 Dec;32(6):1180-92. Abstract

41. Lingman-Framme J, Fasth A. Subcutaneous immunoglobulin for primary and secondary immunodeficiencies: an evidence-based review. Drugs. 2013 Aug;73(12):1307-19. Abstract

42. Quartier P, Debre M, De Blic J, et al. Early and prolonged intravenous immunoglobulin replacement therapy in childhood gammaglobulinemia: a retrospective survey of 31 patients. J Pediatr. 1999 May;134(5):589-96. Abstract

43. de Gracia J, Vendrell M, Alvarez A, et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol. 2004 Jun;4(6):745-53. Abstract

44. Orange JS. Impact of trough IgG on pneumonia incidence in primary immunodeficiency: a meta-analysis of clinical studies. Clin Immunol. 2010 Oct;137(1):21-30. Abstract

45. Myers LA, Patel DD, Puck JM, et al. Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survival. Blood. 2002 Feb 1;99(3):872-8.Full text  Abstract

46. European Medicines Agency. Guideline on core SmPC for human normal immunoglobulin for intravenous administration (IVIg). Jun 2018 [internet publication].Full text

47. European Medicines Agency. Clinical investigation of human normal immunoglobulin for intravenous administration (IVIg). Dec 2021 [internet publication].Full text

48. National Blood Authority, Australia. Criteria for the clinical use of immunoglobulin in Australia: version 3. 2018 [internet publication].Full text

49. Department of Health (UK). Clinical guidelines for immunoglobulin use: second edition update. Aug 2011 [internet publication].Full text

50. Raanani P, Gafter-Gvili A, Paul M, et al. Immunoglobulin prophylaxis in chronic lymphocytic leukemia and multiple myeloma: systematic review and meta-analysis. Leuk Lymphoma. 2009 May;50(5):764-72. Abstract

51. Vacca A, Melaccio A, Sportelli A, et al. Subcutaneous immunoglobulins in patients with multiple myeloma and secondary hypogammaglobulinemia: a randomized trial. Clin Immunol. 2018 Jun;191:110-5. Abstract

52. Bourassa-Blanchette S, Knoll GA, Hutton B, et al. Clinical outcomes of polyvalent immunoglobulin use in solid organ transplant recipients: a systematic review and meta-analysis. Clin Transplant. 2019 Jun;33(6):e13560. Abstract

53. Bourassa-Blanchette S, Patel V, Knoll GA, et al. Clinical outcomes of polyvalent immunoglobulin use in solid organ transplant recipients: a systematic review and meta-analysis - Part II: non-kidney transplant. Clin Transplant. 2019 Jul;33(7):e13625. Abstract

54. Perez EE, Orange JS, Bonilla F, et al. Update on the use of immunoglobulin in human disease: a review of evidence. J Allergy Clin Immunol. 2017 Mar;139(3s):S1-46.Full text  Abstract

55. Wijetilleka S, Jayne DR, Mukhtyar C, et al. Recommendations for the management of secondary hypogammaglobulinaemia due to B cell targeted therapies in autoimmune rheumatic diseases. Rheumatology (Oxford). 2019 May 1;58(5):889-96.Full text  Abstract

56. Wijetilleka S, Mukhtyar C, Jayne D, et al. Immunoglobulin replacement for secondary immunodeficiency after B-cell targeted therapies in autoimmune rheumatic disease: systematic literature review. Autoimmun Rev. 2019 May;18(5):535-41. Abstract

57. Barmettler S, Ong MS, Farmer JR, et al. Association of immunoglobulin levels, infectious risk, and mortality with rituximab and hypogammaglobulinemia. JAMA Netw Open. 2018 Nov 2;1(7):e184169.Full text  Abstract

58. Khojah AM, Miller ML, Klein-Gitelman MS, et al. Rituximab-associated hypogammaglobulinemia in pediatric patients with autoimmune diseases. Pediatr Rheumatol Online J. 2019 Aug 28;17(1):61.Full text  Abstract

59. O'Donohue JM, Enzmann DR. Mycotic aneurysm in angiitis associated with herpes zoster ophthalmicus. AJNR Am J Neuroradiol. 1987 Jul-Aug;8(4):615-9.Full text  Abstract

60. Agarwal S, Cunningham-Rundles C. Treatment of hypogammaglobulinemia in adults: a scoring system to guide decisions on immunoglobulin replacement. J Allergy Clin Immunol. 2013 Jun;131(6):1699-701.Full text  Abstract

61. Mazzatenta C, Martini P, Luti L, et al. Granulomatous dermatitis in common variable immunodeficiency with functional T-cell defect. Arch Dermatol. 2006 Jun;142(6):783-4. Abstract

62. Roifman CM, Schroeder H, Berger M, et al. Comparison of the efficacy of IGIV-C, 10% (caprylate/chromatography) and IGIV-SD, 10% as replacement therapy in primary immune deficiency: a randomized double-blind trial. Int Immunopharmacol. 2003 Sep;3(9):1325-33. Abstract

63. Eades-Perner AM, Gathmann B, Knerr V, et al. The European internet-based patient and research database for primary immunodeficiencies: results 2004-06. Clin Exp Immunol. 2007 Feb;147(2):306-12.Full text  Abstract

64. Liu Z, Albon E, Hyde C. The effectiveness and cost effectiveness of immunoglobulin replacement therapy for primary immunodeficiency and chronic lymphocytic leukaemia: a systematic review and economic evaluation. Birmingham, UK: West Midlands Health Technology Assessment Collaboration, Department of Public Health and Epidemiology, University of Birmingham; 2005:54.Full text

65. Chapel H, Lucas M, Lee M, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008 Jul 15;112(2):277-86.Full text  Abstract

66. Resnick ES, Moshier EL, Godbold JH, et al. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012 Feb 16;119(7):1650-7.Full text  Abstract

67. Pimenta FMCA, Palma SMU, Constantino-Silva RN, et al. Hypogammaglobulinemia: a diagnosis that must not be overlooked. Braz J Med Biol Res. 2019;52(10):e8926.Full text  Abstract

68. Obregon RG, Lynch DA, Kaske T, et al. Radiologic findings of adult primary immunodeficiency disorders. Contribution of CT. Chest. 1994 Aug;106(2):490-5. Abstract

69. Vorechovsky I, Scott D, Haeney MR, et al. Chromosomal radiosensitivity in common variable immune deficiency. Mutat Res. 1993 Dec;290(2):255-64. Abstract

70. Aghamohammadi A, Moin M, Kouhi A, et al. Chromosomal radiosensitivity in patients with common variable immunodeficiency. Immunobiology. 2008;213(5):447-54. Abstract

71. de Albuquerque Campos R, Sato MN, da Silva Duarte AJ. IgG anti-IgA subclasses in common variable immunodeficiency and association with severe adverse reactions to intravenous immunoglobulin therapy. J Clin Immunol. 2000 Jan;20(1):77-82. Abstract

72. Mellemkjaer L, Hammarström L, Andersen V, et al. Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study. Clin Exp Immunol. 2002 Dec;130(3):495-500.Full text  Abstract

73. Kinlen LJ, Webster AD, Bird AG, et al. Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet. 1985 Feb 2;1(8423):263-6. Abstract

74. Cunningham-Rundles C, Siegal FP, Cunningham-Rundles S, et al. Incidence of cancer in 98 patients with common varied immunodeficiency. J Clin Immunol. 1987 Jul;7(4):294-9. Abstract

75. Wang J, Cunningham-Rundles C. Treatment and outcome of autoimmune hematologic disease in common variable immunodeficiency (CVID). J Autoimmun. 2005 Aug;25(1):57-62. Abstract

76. Morimoto Y, Routes JM. Granulomatous disease in common variable immunodeficiency. Curr Allergy Asthma Rep. 2005 Sep;5(5):370-5. Abstract

77. Hartono S, Motosue MS, Khan S, et al. Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2017 May;118(5):614-20. Abstract

78. Mannina A, Chung JH, Swigris JJ, et al. Clinical predictors of a diagnosis of common variable immunodeficiency-related granulomatous-lymphocytic interstitial lung disease. Ann Am Thorac Soc. 2016 Jul;13(7):1042-9.Full text  Abstract

79. Rudge P, Webster AD, Revesz T, et al. Encephalomyelitis in primary hypogammaglobulinaemia. Brain. 1996 Feb;119(Pt 1):1-15.Full text  Abstract

80. Franz A, Webster AD, Furr PM, et al. Mycoplasmal arthritis in patients with primary immunoglobulin deficiency: clinical features and outcome in 18 patients. Br J Rheumatol. 1997 Jun;36(6):661-8.Full text  Abstract

81. Razvi S, Schneider L, Jonas MM, et al. Outcome of intravenous immunoglobulin-transmitted hepatitis C virus infection in primary immunodeficiency. Clin Immunol. 2001 Dec;101(3):284-8. Abstract

82. Md Yusof MY, Vital EM, McElvenny DM, et al. Predicting severe infection and effects of hypogammaglobulinemia during therapy with rituximab in rheumatic and musculoskeletal siseases. Arthritis Rheumatol. 2019 Nov;71(11):1812-23.Full text  Abstract

83. Bonilla FA. Update: vaccines in primary immunodeficiency. J Allergy Clin Immunol. 2018 Feb;141(2):474-81.Full text  Abstract

84. Hammarström L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 2000 May;120(2):225-31.Full text  Abstract

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