Hemophilia in the Orenburg region: analysis of mortality causes, 2008–2024: 616.151.514:314.14/470.56

Abstract

Summary. Introduction. Data on the mortality structure of hemophilia patients are almost absent in the Russian literature. There are some articles devoted to the study of comorbidity in hemophilia. Aim: to evaluate concomitant diseases and determine their contribution to the structure of mortality causes of hemophilia patients in the Orenburg region from 2008 to 2024. Materials and Methods. We conducted a retrospective analysis of all 27 fatal cases with a confirmed diagnosis of congenital FVIII and FIX deficiency, who died in the Orenburg region from January 1, 2008 to December 31, 2024. The information source was data from a personalized register. According to the WHO age classification, the patients were divided into subgroups by age at death: children under 18 years; young adults from 18 to 40 years; middle-aged adults
from 45 to 59 years; older people from 60 to 74 years. Results. The median age at death of hemophilia patients was 46 [34; 55] years; the mean age was 43 (range, 8–64) years that was below the mean age of death of men in the Orenburg region for this period by 23 years. No significant differences in the incidence of fatal cases in the group of young, middle-aged, and older individuals (37.0, 33.3 and 22.2%, respectively)
were obtained. Analysis of concomitant diseases showed that the leading causes in terms of frequency were chronic viral hepatitis (88%) and cardiovascular diseases (56%); less common etiologies included diabetes mellitus (28%), cirrhosis (24%), obesity (24%), and malignant neoplasms (16%), that presented even at a young age. The number of comorbidities per patient increased with age, reaching a maximum in the group of patients aged over 60 years (4.6). The leading causes of death in hemophilia patients were cardiovascular diseases (29.6%), malignant neoplasms (18.5%), digestive diseases (18.5%), injuries and other external factors (14.8%), which is comparable to the mortality structure in the Russian Federation. Conclusion. The obtained results indicate that an expanded screening of hemophilia patients is needed for early detection of concomitant diseases and their timely treatment, including the use of antiviral agents in chronic viral hepatitis C.

For citation: Kuchma G.B., Yakubova U.A., Kozlova L.K., Bagirova G.G., Lebedenko S.A., TatarinovaV.V., Bashkatova T.A., Plotnikova P.A. Hemophilia in the Orenburg region: analysis of mortality causes, 2008–2024. Tromboz, gemostaz i reologiya. 2025;(2):113–121. (In Russ.).

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