Registry of patients with primary immunodeficiency disorders in the Irkutsk region

In this paper, the main epidemiological indicators of primary immunodeficiency disorders (PIDDs) in the Irkutsk region based on analysis of data from 66 patients registered in the PIDDs database from 2010 to November 2021 were studied. The prevalence of primary immunodeficiencies in the Irkutsk region totals 2.75:100,000 of the population. The cohort of living patients is dominated by children (74%). A total of 22 nosological forms were verified in the registry of primary immunodeficiencies. Molecular genetic diagnosis was confirmed in 26% of the patients. Hematopoietic cell transplantation was performed in 21.2% of the patients. Intravenous immunoglobulin preparations are received by 19.7% of patients. The main purpose of the registry is to systematise patients with primary immunodeficiencies, provide them with the necessary treatment in a timely manner and improve their quality of life.