Epidemiological, clinical and treatment aspects of hemophilia in the Republic of Moldova

Abstract

Introduction. Hemophilia is a rare inherited, recessive X-linked disease caused by a deficiency or lack of a plasma coagulation factor (VIII or IX). In the structure of inherited coagulopathies, hemophilia occupies 96-98%, being considered one of the most common forms of coagulopathies. Aim of study. Studying the epidemiological, clinical and treatment aspects of patients with hemophilia in the Republic of Moldova. Methods and materials. Is a retrospective study performed on a group of 92 adult patients with hemophilia, under supervision and treatment in the Hematology Department of the Oncological Institute of the Republic of Moldova. The study is based on the analysis of data from outpatient medical records and clinical observation sheets of the respective patients based on a questionnaire developed to achieve the purpose of the research. The inclusion criteria in the study was the existence of an established clinical diagnosis of hemophilia. Results. As a result of the research, it was determined that the diagnosis of hemophilia A was found in 80 (86.9%) cases, and hemophilia B in 12 (13.1%). The obtained data correspond to those in the literature. Data on the concentration of coagulation factor were also processed, which was determined in only 35 patients, of which 22 (63 %) the concentration was ˂1%, from 1% to 2% - 7 (20 %), from 2% to 5% - 5 (14 %) patient and > 5% in 1 (3 %) cases and the distribution of all patients with hemophilia according to the degree of severity according to clinical data determined the presence of very severe form in 20 (21.7%) patients, severe in 32 (34.8%), moderate in 19 (20.7%) and mild in 21 (22.8%) cases. The analysis of the data regarding the complications of the disease, which is an index of the quality of life of the patient with hemophilia, showed the predominance of the complications of the basic disease (58.7%), mainly arthropathies (54.3%) but it was found that they were diagnosed frequently and post-transfusion. Conclusion. Progress in the treatment of patients with hemophilia, with the introduction of maintenance therapy with factor concentrates, has prevented life-threatening bleeding accidents, reduced the number of hospitalizations, avoided complications that could lead to disability and ensuring a favorable quality of life with a socio-economic impact for a long time.