Email this article 
Primary immunodeficiency - analysis of registry of Institute of Immnunology
- Authors: Latysheva EA1, Latysheva TV1, Pashenkov MV1, Klimova SV1, Manto IA1
-
Affiliations:
- NRC Institute of Immunology FMBA of Russia
- Issue: Vol 15, No 4 (2018)
- Pages: 17-25
- Section: Articles
- URL: https://rusalljournal.ru/raj/article/view/132
- DOI: https://doi.org/10.36691/RJA132
- ID: 132
Cite item
Open Access
Access granted
Abstract
The emergence of highly effective therapy has led to an increase in the life expectancy of patients with primary immunodeficiency (PID). Currently, we have the opportunity to observe the first generation of adults suffering from PID. Objective. To study the structure of the PID forms in Russian adults of the register of Institute of immunology of FMBA Russia. Materials and methods. The analysis of PID structure, demographic data and need for therapy was carried out on the basis of472 patient’s case histories who were treated in the Institute of immunology from 1983 to 2017. Results. The main part of patients older than 18 years (472 people) of Russian PID population are presented with predominant antibody deficiency - 61,22% and hereditary angioedema (HAE) - 30,72%, other PID forms are presented by isolated clinical cases. In 21,82% of cases, the disease debuted at the age of 18 years, with the diagnosis of PID was established after 18 years in 62,4% of cases (294 patients out of472). The average age of adult registry of PID patients is 39,6±6 years, median age - 35,6 years, maximum age - 86 years. Persistent disability is observed in 12,5% of cases. A study of the availability and adequacy of therapy showed that replacement therapy with immunoglobulins (IVIG) in need by 53,7%, but only 27,4% have it on the regularly and conditionally adequate dose, for 5,7% IVIG therapy is unavailable, while the majority of patients - 66,8% receive IVIG irregular and/or in inadequate dose. The presence of splenomegaly, hepatomegaly and enteropathy significantly lengthen the diagnosis period (p<0,05). B-cell phenotyping showed that this method is a specific and reproducible test for the diagnosis of common variable immunodeficiency (CVID), allows to minimize the number of diagnostic errors and reduce the time of diagnosis. The search for reliable immunological markers-predictors of complications of CVID did not give results.
Full Text
About the authors
E A Latysheva
NRC Institute of Immunology FMBA of Russia
Email: ealat@mail.ru
T V Latysheva
NRC Institute of Immunology FMBA of Russia
M V Pashenkov
NRC Institute of Immunology FMBA of Russia
S V Klimova
NRC Institute of Immunology FMBA of Russia
I A Manto
NRC Institute of Immunology FMBA of Russia
References
- Veramendi-Espinoza LE, Zafra-Tanaka JH, Pérez-Casquino GA, Côrdova-Calderôn WO. Diagnostic Delay of Primary Immunodeficiencies at a Tertiary Care Hospital in Peru- Brief Report. J Clin Immunol. 2017;37:383-387.
- Mohammadinejad P, Mirminachi B, Sadeghi B, Movahedi M, Gharagozlou М, Mohammadi J et al. Distribution of primary immunodeficiency disorders diagnosed in a tertiary referral center, Tehran, Iran (2006-2013). Iran J. Immunol. 2014;11:282-291.
- Routes J, Abinun M, Al-Herz W, Bustamante J, Condino-Neto A, De La Morena MT et al. ICON: the early diagnosis of congenital immunodeficiencies. J Clin Immunol. 2014;34:398-424.
- Michs M, Raptaki M, Tantou S, Tzanoudaki M, Spanou K, Liatsis M еt al. Primary immunodeficiency diseases: a 30-year patient registry from the referral center for primary immunodeficiencies in Greece. J Clin Immunol. 2014;34:836-843.
- Mellouli F, Khaled MB, Besbes H, Ouederni M, Mekki N, Ali MB et al. Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012). J Clin Immunol. 2015;35:745-753.
- Timothy C, Aygören-Pürsün E, Bork K, Bowen T, Boysen H, Farkas H et al. WAO Guideline for the Management of Hereditary Angioedema. World Allergy Organ J. 2012;5:182-199.
- Grimbacher B. The European Society for Immunodeficiencies (ESID) registry. Clin Exp Immunol. 2014;178:18-20.
- Gathmann B. Clinical picture and treatment of2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134:116-126.
- Agarwal S. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clin Gastroenterol Hepatol. 2013;11:1050-1063.
- Cowan MJ. The Primary Immune Deficiency Treatment Consortium: how can it improve definitive therapy for PID? Expert Rev Clin Immunol. 2016;12:1007-1009.
- Rousso SZ, Shamriz O, Zilkha A, Braun J, Averbuch D, Or R et al. Hematopoietic Stem Cell Transplantations for Primary Immune Deficiencies: 3 Decades of Experience From a Tertiary Medical Center. J Pediatr Hematol Oncol. 2015;37:295-300.
- Hamidieh AA, Behfar M, Pourpak Z, Faghihi-Kashani S, Fazlollahi MR, Hosseini AS et al. Long-term outcomes of fludarabine, melphalan and antithymocyte globulin as reduced-intensity conditioning regimen for allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiency disorders: a prospective single center study. Bone Marrow Transplant. 2016;51:219-226.
- Wehr С, Gennery AR, Lindemans C, Schulz A, Hoenig M, Marks R et al. Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency. J Allergy Clin Immunol. 2015;135:988-997.
- Edgar JD, Buckland M, Guzman D, Conlon NP, Knerr V, Bangs C et al. The United Kingdom Primary Immune Deficiency (UK-PID) Registry: report of the first 4 years> activity 2008-2012. Clin Exp Immunol. 2014;175:68-78.
- Marschall K, Hoernes M, Bitzenhofer-Grüber M, Jandus P, Duppenthaler A, Wuillemin WA et al. The Swiss National Registry for Primary Immunodeficiencies: report on the first 6 years’ activity from 2008 to 2014. Clin Exp Immunol. 2015;182:45-50.
Supplementary files
