HEMOPHILIA (FROM FATAL OUTCOME TO OUTPATIENT MANAGEMENT)

DOI: https://doi.org/10.29296/25877305-2020-08-05
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Issue: 
8
Year: 
2020

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

Hemophilia is the most common form of severe hereditary bleeding. The prevalence of hemophilia among adolescents according to official statistics in Moscow is 12.2–16.0 per 100 thousand. Hemophilia B occurs 5 times less frequently than hemophilia A, hemophilia C - 10 times less often. Taking into account the localization of the FVIII and FIX genes on the X chromosome and their recessiveness, clinically significant manifestation of hemophilia A and B is characteristic almost only of men. Mild forms of hemophilia are much more common in female carriers than is commonly thought. New female carriers often arise from old fathers (that is, old paternal sperm). Clinically, depending on the degree of clotting factor deficiency (severity of hemophilia), bleeding of varying intensity is possible, up to disabling and life-threatening. Modern replacement therapy with recombinant clotting factors, the appointment of recombinant clotting factors prevents bleeding, allows surgical interventions and ensures a sufficiently high quality of life.
Keywords: 
hemophilia
clinical features
coagulation factors
replacement therapy
Octofactor
Innonafactor
Coagil VII

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