EXPERIENCE IN THE USE OF RITUXIMAB IN THE COMPLEX THERAPY OF PRIMARY IMMUNODEFICIENTS IN ADULTS WITH IMMUNE REGULATION DISORDERS.

Nailya Setdikova; Сетдикова Наиля Харисовна; Evgeny Frolov; Фролов Евгений Александрович; Tatiana Myasnikova; Мясникова Татьяна Николаевна; Tatiana Romanova; Романова Татьяна Сергеевна; Tatiana Latysheva; Латышева Татьяна Васильевна; Elena Latysheva; Латышева Елена Александровна; Gulumhan Aminova; Аминова Гулюмхан Эльвировна; Yuliya Klimanova; Климанова Юлия Михайловна

doi:10.36691/RJA17082

EXPERIENCE IN THE USE OF RITUXIMAB IN THE COMPLEX THERAPY OF PRIMARY IMMUNODEFICIENTS IN ADULTS WITH IMMUNE REGULATION DISORDERS.

Authors: Setdikova N.¹, Frolov E.¹, Myasnikova T.¹, Romanova T.¹, Latysheva T.¹, Latysheva E.¹, Aminova G.¹, Klimanova Y.¹
Affiliations:
1. Federal State Budgetary Institution "State Research Center Institute of Immunology" of the Federal Medical and Biological Agency of Russia
Section: Original studies
Submitted: 11.11.2025
Accepted: 24.03.2026
Published: 16.04.2026
URL: https://rusalljournal.ru/raj/article/view/17082
DOI: https://doi.org/10.36691/RJA17082
ID: 17082

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Abstract

Objective: to analyze the effectiveness of therapy for complications of immune dysregulation in patients with OVIN over 18 years of age using rituximab. Materials and methods: The study included 31 patients aged 19 to 68 years with common variable immune deficiency, manifested by symptoms of immune dysregulation without a detected molecular-genetic defect. Ruximab was included in the complex therapy of the disease for all patients after the stabilization of the condition and adequate saturation with human normal immunoglobulin in order to correct the manifestations of interstitial lung disease and/or splenomegaly and lymphadenopathy and/or thrombocytopenia, and/or autoimmune hemolytic anemia. Results: 31 case histories of patients with OVIN aged from 19 to 62 years were analyzed. The average age of the total sample was 38.13±2.2 years. Of these, 18 (58.06%) were women and 13 (41.94%) were men. All patients showed symptoms of immune dysregulation: IBD was observed in 28 patients (90.32%), including 16 women (88.88%) and 12 men (92.3%). Splenomegaly was observed in all patients who did not have their spleen removed. Splenectomy was performed in 5 patients (16.13%). All patients were men. Lymphadenopathy was observed in 30 patients (96.77%), 12 men (92.31%), and 18 women (100%).Thrombocytopenia was observed in 22 patients of the total sample (70.97%), in 10 men (76.92%) and 12 women (66.67%). Autoimmune hemolytic anemia was observed in 5 patients in the total sample (16.13%), in 3 men (23.07%), 2 women (11.11%). Nineteen patients (60%) had a history of receiving other lines of therapy without effect or with a short-term effect. All patients received Rituximab therapy. The therapy regimen consisted of 4 doses of rituximab at a dose of 375/m2, administered 7 days apart, followed by 3 doses administered every 3 months, and then every 6 months. After one year of treatment, 89.28% of patients with IB had complete regression of both symptoms and changes in instrumental tests.

Keywords

Primary immunodeficiency, rituximab, immune dysregulation, common variable immune deficiency, interstitial lung disease, splenomegaly, lymphadenopathy, cytopenias, and enteropathy.