GENETICALLY DETERMINED DEFICIENCY OF CLOTTING FACTORS AND OPPORTUNITIES OF MODERN SUBSTITUTIONAL THERAPY

DOI: https://doi.org/10.29296/25877305-2021-07-02
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Issue: 
7
Year: 
2021

Professor V. Delyagin, MD Dmitry Rogachev National Medical Research Center for Pediatric
Hematology, Oncology and Immunology, Moscow

Hemophilia A – deficiency of factor (F) VIII, hemophilia B – deficiency of FIX are the most clinically significant and common genetically determined disorders of blood coagulation. Diagnosis is based on the history, nature, location and duration of bleeding and, mainly, on laboratory studies. Typical lengthening of the activated partial thromboplastin time, absence or decrease in the concentration of FVIII for hemophilia A and FIX for hemophilia B. The optimal choice of therapy is the use of recombinant coagulation factors. Domestic recombinant factors Octofactor (rFVIII) and Innonafactor (rFIX) are not inferior to foreign analogues, are characterized by high therapeutic efficacy and low immunogenicity.
Keywords: 
hemophilia
prevalence
clinical picture
diagnosis
treatment

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