Transient Hypogammaglobulinemia of Infancy Medication

  • Author: Alan P Knutsen, MD; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: Mar 26, 2012
 

Medication Summary

Choose antibiotics to cover S pneumoniae, H influenzae, and Moraxella catarrhalis (eg, amoxicillin, second-generation cephalosporins, clarithromycin). Often, prophylactic antibiotics decrease infections. IVIG is rarely needed and is used only when the patient continues to have infections despite antibiotics.

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Immunoglobulins

Class Summary

IVIG or subcutaneous immune globulin is used for antibody replacement therapy.

Immune globulin, intravenous (Carimune NF, Gammagard S/D, Gammagard liquid, Gammar-P, Gammaplex, Gamunex, Optigam, Polygam S/D, Privigen)

 

Purified preparation of gamma globulin derived from large pools of human plasma. Comprises 4 antibody subclasses.

Potential adverse effects include allergic reactions (eg, anaphylaxis, urticaria) because of IgE or anti-IgA antibodies. In a risk-benefit analysis, allergic reactions with IVIG administration in THI probably warrant discontinuation of IVIG. In severe B-cell immunodeficiency diseases in which IVIG is critical to care, premedication with corticosteroids and antihistamines (diphenhydramine) is usually successful in avoiding a reaction. In addition, the different IVIG preparations contain different amounts of IgA. Select an IVIG preparation with the least amount of IgA (eg, Gammagard SD). Contact manufacturer for specific lots low in IgA.

Immune globulin, subcutaneous (Hizentra, Gammagard liquid, Gamunex c)

 

IgG antibodies that neutralize a wide variety of bacterial and viral agents. Neutralizes circulating myelin antibodies through anti-idiotypic antibodies; downregulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade. Peak serum IgG levels are lower and trough IgG levels are higher than those achieved with IVIG. SC administration results in stable steady-state IgG levels when administered weekly. Hizentra is a 20% (ie, 200-mg/mL) SC injectable and Gammagard liquid and Gamunex c are 10% (ie, 100 mg/mL) SC injectables.

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Vaccines

Class Summary

These agents are used to induce active immunity.

Pneumococcal 7-valent conjugate vaccine (Prevnar)

 

Sterile solution of saccharides of capsular antigens of S pneumoniae serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F individually conjugated to diphtheria CRM197 protein. These 7 serotypes have been responsible for >80% of invasive pneumococcal disease in children < 6 years in the United States. Also accounted for 74% of penicillin-nonsusceptible S pneumoniae (PNSP) infections and 100% of pneumococci infections with high-level penicillin resistance. Customary age for first dose is 2 mo, but can be given as young as 6 wk.

Preferred sites of IM injection include the anterolateral aspect of the thigh in infants or deltoid muscle of upper arm in toddlers and young children. Do not inject vaccine in gluteal area or areas where there may be a major nerve trunk or blood vessel.

Number of 0.5 mL doses for series initiated at age 7-11 mo is 3 (4 wk apart; third dose after first birthday), at age 12-23 mo is 2 doses (2 mo apart), for age 2-9 y is one dose.

Minor illnesses, such as a mild upper respiratory tract infection, with or without low-grade fever are not generally contraindications.

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Contributor Information and Disclosures
Author

Alan P Knutsen, MD  Professor of Pediatrics, Director of Pediatric Allergy and Immunology, Director Jeffrey Modell Diagnostic & Research Center for Primary Immuodeficiences (CGCMC), Director of Pediatric Clinical Immunology Laboratory, Department of Pathology, St Louis University Health Sciences Center

Alan P Knutsen, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD  Consulting Staff, Hoffman La Roche Pharmaceuticals

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Dorsey MJ, Orange JS. Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy. Ann Allergy Asthma Immunol. Nov 2006;97(5):590-5. [Medline].

  2. Siegel RL, Issekutz T, Schwaber J, et al. Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N Engl J Med. Nov 26 1981;305(22):1307-13. [Medline].

  3. Dalal I, Reid B, Nisbet-Brown E, Roifman CM. The outcome of patients with hypogammaglobulinemia in infancy and early childhood. J Pediatr. Jul 1998;133(1):144-6. [Medline].

  4. Cano F, Mayo DR, Ballow M. Absent specific viral antibodies in patients with transient hypogammaglobulinemia of infancy. J Allergy Clin Immunol. Feb 1990;85(2):510-3. [Medline].

  5. Kowalczyk D, Mytar B, Zembala M. Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. J Allergy Clin Immunol. Oct 1997;100(4):556-62. [Medline].

  6. Dalal I, Roifman CM. Hypogammaglobulinemia of infancy. Immunol Allergy Clin North Am. 2001;21:129-39.

  7. Tiller TL, Buckley RH. Transient hypogammaglobulinemia of infancy: review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up. J Pediatr. Mar 1978;92(3):347-53. [Medline].

  8. Walker AM, Kemp AS, Hill DJ, Shelton MJ. Features of transient hypogammaglobulinaemia in infants screened for immunological abnormalities. Arch Dis Child. Mar 1994;70(3):183-6. [Medline].

  9. Dressler F, Peter HH, Muller W, Rieger CH. Transient hypogammaglobulinemia of infancy: Five new cases, review of the literature and redefinition. Acta Paediatr Scand. Sep 1989;78(5):767-74. [Medline].

  10. Hayakawa H, Iwata T, Yata J, Kobayashi N. Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol. Jan 1981;1(1):31-9. [Medline].

  11. Wolpert J, Knutsen AP. Natural history of selective antibody deficiency to bacterial polysaccharide antigens in children. Pediatr Asthma, Allergy, Immunol. 1998;12:183-191.

  12. Whelan MA, Hwan WH, Beausoleil J, et al. Infants presenting with recurrent infections and low immunoglobulins: characteristics and analysis of normalization. J Clin Immunol. Jan 2006;26(1):7-11. [Medline].

  13. Ji-hong Q, Jian-xing Z, Xiao-dong Z, Tong-xin C. Clinical features and follow-up of Chinese patients with symptomatic hypogammaglobulinemia in infancy. Chin Med J. 2009;122:1877-1883. [Medline].

  14. Hsueh KC, Chiu HH, Lin HC, et al. Transient hypogammaglobulinemia of infancy presenting as Staphylococcus aureus sepsis with deep neck infection. J Microbiol Immunol Infect. Apr 2005;38(2):141-4. [Medline].

  15. Bukowska-Strakova, Kowalczyk D, Baran J, Siedlar M, Kobylarz K, Zembala M. The B-cell compartment in the peripheral blood of children with different types of primary immunodeficiency. Pediatr Res. 2009;66:28-34. [Medline].

  16. Moschese V, Carsetti R, Graziani L, Chini L, Soresina AR, La Rocca M, et al. Memory B-cell subsets as a predictive marker outcome in hypogammaglobulinemia during infancy. J Allergy Clin Immunol. August 2007;120:474-476. [Medline].

  17. Moschese V, Graziani L, Avanzini MA, Carsetti R, Marconi M, La Rocca M, et al. A prospective study on children with initial diagnosis of transient hypogammaglobulinemia of infancy: results from the Italian Primary Immunodeficiency Network. Inter J Immunopath Pharmacol. April-June2008;21:343-352. [Medline].

  18. Kuijpers TW, Bende RJ, Baars PA, Grummels A, Derks IA, Dolman KM, et al. CD20 deficiency in humans results in impaired T cell-independent antibody responses. J Clin Invest. December 2010;120:214-222. [Medline].

  19. van Zelm MC, Smet J, Adams B, Mascart F, Schandené L, Janssen F, et al. CD81 gene defect in humans disrupts CD19 complex formation and leads to antibody deficiency. J Clin Invest. April 2010;120:1265-1274. [Medline].

  20. Thiel J, Kimmig L, Salzer U, Grudzien M, Lebrecht D, Hagena T, et al. Genetic CD21 deficiency is associated with hypogammaglobulinemia. J Allergy Clin Immunol. March 2012;129:801-810. [Medline].

  21. Sorensen RU, Leiva LE, Giangrosso PA, et al. Response to a heptavalent conjugate Streptococcus pneumoniae vaccine in children with recurrent infections who are unresponsive to the polysaccharide vaccine. Pediatr Infect Dis J. Aug 1998;17(8):685-91. [Medline].

  22. Castigli E, Wilson SA, Scott S, et al. TACI and BAFF-R mediate isotype switching in B cells. J Exp Med. Jan 3 2005;201(1):35-9. [Medline].

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

  24. Dogu F, Ikinciogullari A, Babacan E. Transient hypogammaglobulinemia of infancy and early childhood: outcome of 30 cases. Turk J Pediatr. Apr-Jun 2004;46(2):120-4. [Medline].

  25. Kowalczyk D, Baran J, Webster AD, Zembala M. Intracellular cytokine production by Th1/Th2 lymphocytes and monocytes of children with symptomatic transient hypogammaglobulinaemia of infancy (THI) and selective IgA deficiency (SIgAD). Clin Exp Immunol. Mar 2002;127(3):507-12. [Medline].

  26. Mayer L, Posnett DN, Kunkel HG. Human malignant T cells capable of inducing an immunoglobulin class switch. J Exp Med. Jan 1 1985;161(1):134-44. [Medline].

  27. McGeady SJ. Transient hypogammaglobulinemia of infancy: need to reconsider name and definition. J Pediatr. Jan 1987;110(1):47-50. [Medline].

  28. Oxelius VA. IgG subclass pattern in primary immunodeficiency disorders. Monogr Allergy. 1986;19:156-63. [Medline].

  29. Salzer U, Maul-Pavicic A, Cunningham-Rundles C, et al. ICOS deficiency in patients with common variable immunodeficiency. Clin Immunol. Dec 2004;113(3):234-40. [Medline].

  30. Sneller MC. Common variable immunodeficiency. Am J Med Sci. Jan 2001;321(1):42-8. [Medline].

  31. Sneller MC, Strober W, Eisenstein E, et al. NIH conference. New insights into common variable immunodeficiency. Ann Intern Med. May 1 1993;118(9):720-30. [Medline].

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