• Modern approach to diagnostic and treatment of primary antibody deficiency 
To content

Modern approach to diagnostic and treatment of primary antibody deficiency 

SOVREMENNAYA PEDIATRIYA.2015.6(70):102-109; doi 10.15574/SP.2015.70.102 

Modern approach to diagnostic and treatment of primary antibody deficiency 

Volokha A. P.

Shupyk National Medical Academy of Postgraduate Education, Kyiv, Ukraine 

Primary antibody deficiencies — the largest group of congenital immunodeficiencies. This clinically and genetically heterogeneous group of congenital immune defects includes mild, often asymptomatic IgA deficiency and IgG subclasses and severe agammaglobulinemia with inhibition of production of all immunoglobulines. The most common clinical manifestation — severe or recurrent synopulmonary infections. It is possible the development of autoimmune diseases, enteropathy. Replacement therapy with immunoglobulin — the main method of treatment of hypogammaglobulinemia. 

Key worlds: primary antibody deficiency, diagnostic, treatment. 


1. Cant A, Slatter M, Battersby A. 2013. Advances in management of primary immunodeficiency. Paediatrics and Child Health. 23(3): 115—120. http://dx.doi.org/10.1016/j.paed.2013.02.001

2. Chapel H, Prevot J, Gaspar B. 2014. Primary immune deficiencies — principles of care. Frontiers in Immunology. 5; Article 627. http://dx.doi.org/10.3389/fimmu.2014.00627

3. Tarzi MD, Grigoriadou S, Carr SB et al. Clinical Immunology Review Series: An approach to the management of pulmonary disease in primary antibody deficiency. Clinical and Experimental Immunology. 155: 147—155. http://dx.doi.org/10.1111/j.1365-2249.2008.03851.x; PMid:19128358 PMCid:PMC2675244

4. Gathmann B, Mahlaoui N for CEREDIH, Gerard L et al. 2014. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. http://dx.doi.org/10.1016/j.jaci.2013.12.1077; PMid:24582312

5. Conley ME, Dobbs K, Farmer DM. 2009. Primary B Cell Immunodeficiencies: Comparisons and Contrasts. Annu Rev Immunol. 27: 199—227. http://dx.doi.org/10.1146/annurev.immunol.021908.132649; PMid:19302039

6. Cunningham-Rundles C. 2012.The many faces of common variable immuno-deficiency. Hematology. PMid:23233596 PMCid:PMC4066657

7. Driessen G, van der Burg M. 2011. Primary antibody deficiencies. Educational paper. Eur J Pediatr. 170: 693—702. http://dx.doi.org/10.1007/s00431-011-1474-x; PMid:21544519 PMCid:PMC3098982

8. Durandy A, Kracker S, Fischer A. 2013.Primary antibody deficiencies. Nature Reviews Immunology. http://dx.doi.org/10.1038/nri3466; PMid:23765059

9. Quinti I, Soresina A, Guerra A et al. 2011. Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study. J Clin Immunol. 31: 315—322. http://dx.doi.org/10.1007/s10875-011-9511-0; PMid:21365217

10. Fried AJ, Bonilla FA. 2009, July. Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections. Clinical Microbiology Reviews: 396—414. http://dx.doi.org/10.1128/CMR.00001-09; PMid:19597006 PMCid:PMC2708392

11. Herriot R, Sewell WAC. 2008. Antibody deficiency. J Clin Pathol. 61: 994—1000. http://dx.doi.org/10.1136/jcp.2007.051177; PMid:18755724

12. Kuruvilla M, de la Morena MT. 2013. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract. 1: 573—82. http://dx.doi.org/10.1016/j.jaip.2013.09.013; PMid:24565703

13. Loubser M. 2005. A practical approach to antibody deficiency syndromes. Current Allergy & Clinical Immunology. 4;4: 169—174. http://dx.doi.org/10.1007/s00018-011-0836-x; PMid:22042269

14. Van der Burg M, van Zelm MC, Driessen G, van Dongen JJM. 2012. New frontiers of primary antibody deficiencies. Cell Mol Life Sci. 69: 59—73.

15. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. 2012. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 119;7. http://dx.doi.org/10.1182/blood-2011-09-377945; PMid:22180439 PMCid:PMC3286343

16. Aguilar C, Malphettes M, Donadieu J et al. 2015. Prevention of infections during primary immunedeficiencies. Clinical Infectious Diseases. http://cid.oxfordjournals.org.

17. Al-Herz W, Bousfiha A, Casanova J-L et al. 2014. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. Frontiers in Immuunology. 5.

18. Tam JS, Routes JM. 2013.Common variable immunodeficiency. Am J Rhinol Allergy. 27: 260—265. http://dx.doi.org/10.2500/ajra.2013.27.3899; PMid:23883805 PMCid:PMC3901442

19. Orange JS, Ballow M, Stiehm R et al. 2012. Use and interpretation of diagnostic vaccination in primary immunodeficiency: A working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol. 130: 1—24. http://dx.doi.org/10.1016/j.jaci.2012.07.002; PMid:22935624

20. Wood P. 2012. Human normal immunoglobulin in the treatment of primary immunodeficiency diseases. Therapeutics and Clinical Risk Management. 8: 157—167. http://dx.doi.org/10.2147/TCRM.S22599; PMid:22547934 PMCid:PMC3333462

21. Wood P. 2009. On behalf of a working party of the UK Primary Immunodeficiency Network Primary antibody deficiencies: recognition, clinical diagnosis and referral of patients. Clinical Medicine. 9;6: 595—9. http://dx.doi.org/10.7861/clinmedicine.9-6-595

22. Yel L. 2010. Selective IgA Deficiency. J Clin Immunol. 30: 10—16. http://dx.doi.org/10.1007/s10875-009-9357-x; PMid:20101521 PMCid:PMC2821513

23. Yong PFK, Chee R, Grimbacher B. 2008. Hypogammaglobulinaemia. Immunol Allergy Clin N Am. 28: 691—713. http://dx.doi.org/10.1016/j.iac.2008.06.003; PMid:18940570