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Registration Number

0223U000497, 0121U100287 , R & D reports

Title

To Improve prevention of the occurrence of inhibitor in hemophilia patients by creating a prognostic model, based on the study of risk factors

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Head

Novak Vasyl L., Доктор медичних наук
Stasyshyn Oleksandra V., Доктор медичних наук

Registration Date

09-01-2023

Organization

State institution "Institute of blood pathology and transfusion medicine of Nataional Academy of Medical scinces of Ukraine"

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The object is 657 patients with hemophilia A and B, who were treated in the SI “IPKTM of UNAMS”. The goal is to develop an adequate model, convenient for use in practical medicine, based on the study of risk factors, in order to predict and prevent of an inhibitors in hemophilia patients. Research methods are clinical, cytological, hematological, biochemical, coagulological and molecular genetic. Discrete choice regression models were used to substantiate the study of the relationship between potential risk factors and the probability of developing an inhibitors. It was found that in patients with a permanent inhibitor, the combined genotype АГ –1082 IL-10 /AG –308 TNFα is detected less often and the genotype GG –1082 IL-10 /GG –308 TNFα is more common. In hemophilia complicated by an inhibitors, the frequency of the A-allele and the AA-genotype of SNP +49 A>G exon 1 of the CTLA-4 gene is higher compared to patients without an inhibitor. The combined TT/AA genotype occurs more often, and in the presence of a permanent inhibitor, the CC/AG genotype is significantly less common compared to patients with a transient inhibitor. Alleles DRB1*1501-1502, DRB1*0701-0702, and DQВ1*0602 can be considered as “aggressor” alleles that increase the risk of the appearance of an inhibitor, and the DQВ1*0302 allele, on the contrary, as a “protector” allele that reduces this risk. Carriership of the DRB1*1501-1502 and DQВ1*0602 allelic group increases the risk of inhibitor development in hemophilia patients tenfold (OR=10.7; CI 95%: 0.58-198.91) each, and, conversely, DQВ1*0302 carriership in five times reduces the risk of developing an inhibitor (OR=5.5; CI 95%: 1.29-23.17). In 23 (46%) patients with a severe hemophilia A, "large" mutations were detected, but no inhibitor was detected. By the method of multiple logit regressions, 2 predictors of the appearance of an inhibitor in hemophilia patients were identified: patient age and family inhibitor history.

Product Description

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Dzis Roman Petroovych
Krasivska Valeria Valeriyivna
Savulak Galyna Romanivna

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2023-01-09