Hypogammaglobulinemia Questions & Answers

1. Buckley R. Primary immunodeficiency diseases. Middleton E Jr, Reed CE, Ellis EF, Adkinson NF Jr, Yunginger JW, Busse WW, eds. Allergy: Principles & Practice. 5th ed. Mo: Mosby: St. Louis; 1998. 713-34.

2. Ochs HD, Smith CIE, Puck JM. Primary Immunodeficiency Diseases: A Molecular & Cellular Approach. 2nd ed. USA: Oxford University Press; 2006.

3. Sneller MC. Common variable immunodeficiency. Am J Med Sci. 2001 Jan. 321(1):42-8. [QxMD MEDLINE Link].

4. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999 Jul. 92(1):34-48. [QxMD MEDLINE Link].

5. Conley ME, Howard V. Clinical findings leading to the diagnosis of X-linked agammaglobulinemia. J Pediatr. 2002 Oct. 141(4):566-71. [QxMD MEDLINE Link].

6. Samson M, Audia S, Lakomy D, Bonnotte B, Tavernier C, Ornetti P. Diagnostic strategy for patients with hypogammaglobulinemia in rheumatology. Joint Bone Spine. 2011 May. 78(3):241-5. [QxMD MEDLINE Link].

7. Paris K, Sorensen RU. Assessment and clinical interpretation of polysaccharide antibody responses. Ann Allergy Asthma Immunol. 2007 Nov. 99(5):462-4. [QxMD MEDLINE Link].

8. Chouksey AK, Berger M. Assessment of protein antibody response in patients with suspected immune deficiency. Ann Allergy Asthma Immunol. 2008 Feb. 100(2):166-8. [QxMD MEDLINE Link].

9. Barroeta Seijas AB, Graziani S, Cancrini C, Finocchi A, Ferrari S, Miniero R, et al. The impact of TACI mutations: from hypogammaglobulinemia in infancy to autoimmunity in adulthood. Int J Immunopathol Pharmacol. 2012 Apr-Jun. 25(2):407-14. [QxMD MEDLINE Link].

10. Buckley R, ed. Immune Deficiency Foundation Diagnostic and Clinical Care Guidelines for Primary Immunodeficiency Diseases, 2nd ed. 2009. Accessed August 17, 2009. Immune Deficiency Foundation. [Full Text].

11. Boztug K, Dewey RA, Klein C. Development of hematopoietic stem cell gene therapy for Wiskott-Aldrich syndrome. Curr Opin Mol Ther. 2006 Oct. 8(5):390-5. [QxMD MEDLINE Link].

12. Raanani P, Gafter-Gvili A, Paul M, Ben-Bassat I, Leibovici L, Shpilberg O. Immunoglobulin prophylaxis in chronic lymphocytic leukemia and multiple myeloma: systematic review and meta-analysis. Leuk Lymphoma. 2009 May. 50(5):764-72. [QxMD MEDLINE Link].

13. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005. 94(5 Suppl 1):S1-63. [QxMD MEDLINE Link].

14. Mechanic LJ, Dikman S, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Intern Med. 1997 Oct 15. 127(8 Pt 1):613-7. [QxMD MEDLINE Link].

15. Kainulainen L, Varpula M, Liippo K, Svedstrom E, Nikoskelainen J, Ruuskanen O. Pulmonary abnormalities in patients with primary hypogammaglobulinemia. J Allergy Clin Immunol. 1999 Nov. 104(5):1031-6. [QxMD MEDLINE Link].

16. Sokolic R, Kesserwan C, Candotti F. Recent advances in gene therapy for severe congenital immunodeficiency diseases. Curr Opin Hematol. 2008 Jul. 15(4):375-80. [QxMD MEDLINE Link]. [Full Text].

17. Aiuti A, Cattaneo F, Galimberti S, Benninghoff U, Cassani B, Callegaro L, et al. Gene therapy for immunodeficiency due to adenosine deaminase deficiency. N Engl J Med. 2009 Jan 29. 360(5):447-58. [QxMD MEDLINE Link].

18. [Guideline] Kobayashi M, Bennett NM, Gierke R, et al. Intervals Between PCV13 and PPSV23 Vaccines: Recommendations of the Advisory Committee on Immunization Practices (ACIP). Morbidity and Mortality Weekly Report. 2015 Sept 04. Vol 64:944-47. [Full Text].

19. Orange JS, Hossny EM, Weiler CR, et al; Primary Immunodeficiency Committee of the AAAAI. Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol. 2006 Apr. 117(4 Suppl):S525-53. [QxMD MEDLINE Link].

20. Berger M. Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency. Immunol Allergy Clin North Am. 2008 May. 28(2):413-37, x. [QxMD MEDLINE Link].

21. Artac H, Kara R, Gokturk B, Reisli I. Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy. Clin Exp Med. 2012 Jul 21. [QxMD MEDLINE Link].

22. Ballow M, O'Neil K. Approach to the patient with recurrent infections. Middleton E Jr, Reed CE, Ellis EF, Adkinson NF Jr, Yunginger JW, Busse WW, eds. Allergy: Principles & Practice. 5th ed. Mo: Mosby: St. Louis; 1998.

23. Bjoro K, Haaland T, Skaug K. The spectrum of hepatobiliary disease in primary hypogammaglobulinaemia. J Intern Med. 1999 May. 245(5):517-24. [QxMD MEDLINE Link].

24. Bonilla FA, Geha RS. Primary immunodeficiency diseases. J Clin Immunol. 2003. 111(2 Suppl):S571-81. [QxMD MEDLINE Link].

25. Bonilla FA, Geha RS. Update of primary immunodeficiency disease. J Clin Immunol. 2006. 117(2):S435-41. [QxMD MEDLINE Link].

26. Carrol WL, Korsmeyer SJ. Immunoglobulins. Frank M, Austen K, Claman H, Unanue E, eds. Samter's Immunologic Diseases. 5th ed. Little, Brown and Company: Boston, Mass; 1995. 33-51.

27. Casulo C, Maragulia J, Zelenetz AD. Incidence of Hypogammaglobulinemia in Patients Receiving Rituximab and the Use of Intravenous Immunoglobulin for Recurrent Infections. Clin Lymphoma Myeloma Leuk. 2012 Dec 28. [QxMD MEDLINE Link].

28. Cavazzana-Calvo M, Hacein-Bey S, de Saint Basile G, Gross F, Yvon E, Nusbaum P, et al. Gene therapy of human severe combined immunodeficiency (SCID)-X1 disease. Science. 2000 Apr 28. 288(5466):669-72. [QxMD MEDLINE Link].

29. Cooper N, Davies EG, Thrasher AJ. Repeated courses of rituximab for autoimmune cytopenias may precipitate profound hypogammaglobulinaemia requiring replacement intravenous immunoglobulin. Br J Haematol. 2009 Jun. 146(1):120-2. [QxMD MEDLINE Link].

30. Latiff AH, Kerr MA. The clinical significance of immunoglobulin A deficiency. Ann Clin Biochem. 2007 Mar. 44(Pt 2):131-9. [QxMD MEDLINE Link].

31. Li James TC. Immunoglobulin structure and function. Middleton E Jr, Reed CE, Ellis EF, Adkinson NF Jr, Yunginger JW, Busse WW, eds. Allergy: Principles & Practice. 5th ed. Mo: Mosby: St. Louis; 1998. 46-57.

32. Pasternack M. Approach to the adult with recurrent infections. UpToDate. Available at http://www.uptodate.com. Accessed: October 8, 2007.

33. Priary Immunodeficiency Resource Center. National Primary Immunodeficiency Resource Center. Available at http://info4pi.org. Accessed: October 8, 2007.

34. Smith JK, Krishnaswamy GH, Dykes R, Reynolds S, Berk SL. Clinical manifestations of IgE hypogammaglobulinemia. Ann Allergy Asthma Immunol. 1997 Mar. 78(3):313-8. [QxMD MEDLINE Link].

35. Stiehm ER. Immunologic Disorders in Infants and Children. 4th ed. WB Saunders Co; 1996.

36. Tsiodras S, Samonis G, Keating MJ, Kontoyiannis DP. Infection and immunity in chronic lymphocytic leukemia. Mayo Clin Proc. 2000 Oct. 75(10):1039-54. [QxMD MEDLINE Link].

37. World Health Organization Scientific Group. Primary immunodeficiency diseases. Report of a WHO scientific group. Clin Exp Immunol. 1997 Aug. 109 Suppl 1:1-28. [QxMD MEDLINE Link].

Elizabeth A Secord, MD Associate Training Program Director, Allergy and Immunology Fellowship Program, Associate Professor (Clinical-Educator), Department of Pediatrics, Division of Pediatric Immunology, Wayne State University School of Medicine

Elizabeth A Secord, MD 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 Medical Association

Disclosure: Nothing to disclose.

Milind Pansare, MBBS, MD Clinical Assistant Professor, Department of Pediatrics, Division of Allergy/Immunology, Children's Hospital of Michigan, Wayne State University School of Medicine

Milind Pansare, MBBS, MD is a member of the following medical societies: American Academy of Allergy, Asthma and Immunology, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

Divya Seth, MD Assistant Professor in Pediatric Allergy and Immunology, Department of Pediatrics, Wayne State University School of Medicine; Clinical Educator, Children’s Hospital of Michigan

Divya Seth, MD 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

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.

Robert Y Lin, MD Professor, Department of Medicine, New York Medical College; Chief, Allergy and Immunology, and Director of Utilization Review, Department Medicine, New York Downtown Hospital

Robert Y Lin, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, New York Allergy & Asthma Society

Disclosure: Nothing to disclose.

Jenny Shliozberg, MD Associate Clinical Professor, Department of Pediatrics, Division of Allergy and Immunology, Albert Einstein College of Medicine; Consulting Staff, Department of Pediatrics, Montefiore Hospital Medical Center and Albert Einstein College of Medicine; Director of Pediatric Allergy and Immunization Clinic, Children's Hospital at Montefiore Medical Center

Jenny Shliozberg, MD 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, International AIDS Society

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.

Amit J Shah, MD Allergist/Immunologist, Asthma and Allergy Clinic of Utah, Salt Lake City, UT

Amit J Shah, 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.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors James O Ballard, MD, Issam Makhoul, MD, and Avi M Deener, MD, to the development and writing of this article.

• Print this section
• Print the entire contents of
• Print the entire contents of article