PROGNOSTIC CHANGES IN LYMPHOCYTE SUBPOPULATIONS DURING THE DEVELOPMENT OF AUTOIMMUNE COMPLICATIONS IN PATIENTS WITH DIGEORGE SYNDROME
- Authors: Davydova N.V.1, Zinovieva N.V.1, Zimin S.B.2, Shvez O.V.3, Galeeva E.V.1, Molochnikova O.V.1, Petrova J.V.1, Konoplyannikova J.E.1, Gildeeva G.N.4, Kozlov I.G.4
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Affiliations:
- G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
- Morozov City Children’s Hospital Department of Health of Moscow, Moscow, Russian Federation
- Polyclinic № 3 of JSC "Family Doctor", Moscow, Russian Federation
- IPDE of I.M. Sechenov 1st MSMU of the MOH of Russia (SechenovUniversity), Moscow, Russian Federation
- Section: Joint Immunology Forum 2024
- URL: https://rusimmun.ru/jour/article/view/16947
- DOI: https://doi.org/10.46235/1028-7221-16947-PCI
- ID: 16947
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Abstract
AbstractPatients with 22q11.2 deletion syndrome (DiGeorge syndrome) are characterized by a combination of a wide range of pediatric problems with an immunodeficiency. Defects are characterized by T-cell lymphopenia, changes in the functions and subpopulation composition of T and B lymphocytes. Disturbances in lymphocyte homeostasis can lead not only to severe infectious diseases, but also to autoimmune complications, especially in older children. The purpose of this study was to compare the subpopulations of T and B lymphocytes with and without autoimmune complications and to search for prognostic signs that precede the development of complications. The study included 20 patients aged 10 to 18 years with a confirmed diagnosis of DiGeorge syndrome. The patients were divided into 2 groups, according to the presence and absence of autoimmune complications. Subpopulations of lymphocytes were assessed by flow cytometry. No statistically significant differences were found between CD3 T lymphocytes, CD4 T helper, CD8 T cytotoxic and subpopulations of T helper (p>0.05). However, in the group of patients with autoimmune complications, a statistically significant decrease in CD45RA+ naïve T-helper cells was detected, both in relative (p=0.02) and absolute number (p=0.025) and regulatory T cells (respectively, p=0.02 and p=0.007). Among B-lymphocyte in patients with autoimmune complications, a decrease in memory B cells in relative (p = 0.031) and absolute number (p = 0.005) and switched memory (p = 0.016 and p = 0.031) was detected. But transitional B lymphocytes, on the contrary, were increased in relative quantity (p = 0.003). There were no differences between the groups in the level of plasmablasts, activated B-lymphocytes CD21low CD38low, IgM only B-cells (p>0.05). The ROC analysis showed that the most diagnostically and prognostically significant indicators are the relative number of CD45 RA+ naive T cells (cut-off ≤ 28.7%), switched memory B cells - relative (cut-off ≤ 5.0 %) and the absolute number (cut-off ≤ 11) and the relative number of transitional B cells (cut-off ≤ 12.9%). Our data confirm the important role of regular immunophenotyping and especially subpopulations of CD45 RA+ naive T cells, switched memory B cells and transitional B cells in predicting autoimmune complications in this category of patients.
About the authors
Nataliia Vladimirovna Davydova
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Email: nata1902@yandex.ru
ORCID iD: 0000-0002-7325-6045
SPIN-code: 9997-6197
Scopus Author ID: 57195245318
PhD, Doctor of Clinical Laboratory Diagnostics of the Laboratory Diagnostic Department
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Natalia Valentinovna Zinovieva
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Email: nvzinov@gmail.com
ORCID iD: 0000-0002-6926-2055
PhD, Allergist-Immunologist, Head of the Department of Allergology and Immunology №1
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Sergey Borisovich Zimin
Morozov City Children’s Hospital Department of Health of Moscow, Moscow, Russian Federation
Email: zimin-sb@rambler.ru
ORCID iD: 0000-0002-4514-8469
Pediatrist, Нead of the Pediatric Somatic Department
Russian Federation, 119049, Russia, Moscow, 4th Dobryninsky Lane, 1/9Olesya Vasilievna Shvez
Polyclinic № 3 of JSC "Family Doctor", Moscow, Russian Federation
Email: olesyashvets977@hotmail.com
ORCID iD: 0009-0007-9728-3834
PhD, Pediatrist, Deputy Chief Physician
Russian Federation, 115563, Russia, Moscow, Borisovsky proezd, 9 AElena Valentinovna Galeeva
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Email: elengaleeva@yandex.ru
ORCID iD: 0000-0003-1307-3463
Biologist, Head of the Laboratory Diagnostic Department
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Olga Vasilievna Molochnikova
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Email: vasilich_2000@mail.ru
ORCID iD: 0009-0007-0746-9484
Pediatrist of the Consultative and Diagnostic Center
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Julia Victorovna Petrova
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Email: petra200@yandex.ru
ORCID iD: 0009-0000-3356-3633
Pediatrist of the Department of Allergology and Immunology №1
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Julia Engenievna Konoplyannikova
G.N. Speransky City Children’s Hospital № 9 Department of Health of Moscow, Moscow, Russian Federation
Author for correspondence.
Email: phdimmunology@mail.ru
ORCID iD: 0009-0000-7635-0144
Ph.D., Allergist-Iimmunologist, Head of the Center for Allergology and Immunology
Russian Federation, 123317, Russia, Moscow, Shmitovsky proezd, 29Geliya Njazyfovna Gildeeva
IPDE of I.M. Sechenov 1st MSMU of the MOH of Russia (SechenovUniversity), Moscow, Russian Federation
Email: gildeeva_g_n@staff.sechenov.ru
ORCID iD: 0000-0002-2537-2850
MD, PhD, Prof., Head of the Management and Organization of Drug Supply Chair
Russian Federation, 119991, Russia, Moscow, st. Trubetskaya, 8, building 2Ivan Genrihovich Kozlov
IPDE of I.M. Sechenov 1st MSMU of the MOH of Russia (SechenovUniversity), Moscow, Russian Federation
Email: immunopharmacology@yandex.ru
ORCID iD: 0000-0002-9694-5687
MD, PhD, Prof., Prof. of the Management and Organization of Drug Supply Chair
Russian Federation, 119991, Russia, Moscow, st. Trubetskaya, 8, building 2References
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