Optimization of Therapy of Patient with Genetic Defect Antibody Production

Cover Page


Development of molecular genetics and immunological diagnostics, disclosure of pathogenesis of various forms of primary immunodeficiency (PID), improvement of therapeutic techniques contributed to the formation of a generation patients with PID older than 18 years. Experience shows that isolated replacement therapy, leading to a reduction in the frequency and severity of reactivation chronic foci of infection, is not able in some cases to fully stabilize the condition of patients, and the use of antibiotics remains inevitable. Interesting is the development and implementation of the concept of combined therapy of PID humoral therapy, the ideology of which is based on the combination of substitution therapy with exposure on the functional potential of the cellular link of the immune system. One of the perspective variants of influence on functions of cellular components of immune is the use of innate immunity receptor agonists. From the position of the specific clinical application deserves the attention medication Likopid (glucosaminilmuramyl dipeptide) - semi-synthetic analogue of muramyldipeptide, minimally biologically active substance peptidoglycan fragment of the cell wall of all known bacteria. Presented clinical case of X-linked agammaglobulinemia. The patient, despite regular replacement therapy with intravenous immunoglobulins with maintaining a pretransfusion IgG level of at least 8 g/l, continued to register episodes of exacerbation of chronic conjunctivitis up to 10 times a year. Application of licopid in addition to IVIG therapy contributed to the improvement quality of life, reducing the frequency of exacerbations, conjunctivitis and duration of use antibiotics. This example illustrates the role of cellular components innate immune response in clinical manifestation of insufficiency humoral link adaptive immunity, and confirms the possibility of additional correction of these parameters to improve the efficiency of the standard substitution therapy.

About the authors

L. P. Sizyakina

Rostov State Medical University

Author for correspondence.
Email: iai3012@rambler.ru
Rostov-on-Don, Russian Federation

I. I. Andreeva

Rostov State Medical University

Email: iai3012@rambler.ru
Rostov-on-Don, Russian Federation

S. V. Petruchik

City Hospital № 7

Email: iai3012@rambler.ru
Taganrog, Russian Federation


  1. Sizyakina LP, Andreeva II. Creating a patient regis­ter as an effective tool for the diagnosis of primary immunodeficiencies. Pediatric pharmacology. 2013; 10(5): 94—6. (In Russ.)
  2. Latysheva EA, Latysheva TV, Pashchenkov MV. Primary immunodeficiencies in adults — an analysis of the register of the Institute of Immunology. Russian Allergological Journal. 2018;15(9): 17—26. (In Russ.)
  3. Chapel H. Common variable immunodeficiency disorders (CVID) — diagnoses of exclusion, especially combined immune defects. J Allergy Clin Immunol Pract. 2016;4: 1158—9.
  4. Shillitoe B, Gennery A. X-Linked Agammaglobulinaemia: Outcomes in the modern era. Clinical Immunology. 2017;183: 54—62.
  5. Quinti I, Soresina A, Spadaro G. et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27: 308—16.
  6. Pittet LA, Hall-Stoodley L, Rutkowski MR, Harmsen AG. Influenza virus infection decreases tracheal mucociliary velocity and clearance of Streptococcus pneumonia. Am J Respir Cell Mol Biol. 2010;42: 450—60.
  7. McNamee LA, Harmsen AG. Both influenza-induced neutrophil dysfunction and neutrophil-independent mechanisms contribute to increased susceptibility to a secondary Streptococcus pneumoniae infection. Infect Immun. 2006;74: 6707—21.
  8. Hament JM, Kimpen JL, Fleer A,Wolfs TF. Respiratory viral infection predisposing for bacterial disease: a concise review. FEMS Immunol Med Microbiol. 1999;26: 189—95.
  9. Baumann U, Miescher S, Vonarburg C. Immunoglobulin replacement therapy in antibody deficiency syndromes: are we really doing enough. Clinical and Experimental Immunology. 2014;178: 83—5.
  10. Sizyakina LP, Andreeva II, Krolevets DI. Functional activity of cellular factors of innate immunity in pri­mary agammaglobulinemia. Immunology. 2017;38(4): 223—6. (In Russ.)
  11. Sizyakina L.P., Andreeva I.I., Krolevets D.I. A me­thod for evaluating the effectiveness of treatment of patients with generally variable immune deficiency. Russian Federation Patent № 2683242: 27.03.2019. Newsletter 2.



Abstract - 279

PDF (Russian) - 267




Copyright (c) 2019 Sizyakina L.P., Andreeva I.I., Petruchik S.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies