Terapevticheskii arkhivTerapevticheskii arkhiv0040-36602309-5342LLC Obyedinennaya Redaktsiya3213010.17116/terarkh20168812120-125Review ArticleHereditary afibrinogenemia: A literature review and clinical observationsYakovlevaE V-SurinV L-SelivanovaD S-SergeevaA M-GonсharovaM V-DemidovaE Yu-SobolevaN P-MakhinyaS A-DezhenkovaA V-LikhachevaE A-ZozulyaN I-15122016881212012510042020Copyright © 2016, Consilium Medicum2016Afibrinogenemia is a rare congenital coagulopathy that leads to life-threatening bleeding. In afibrinogenemia, plasma fibrinogen levels are less than 0.1 g/L. The clinical manifestations of the disease can be both bleeding and thromboses of different localizations, which is determined by the multifunctional role of fibrinogen in hemostasis. The described cases demonstrate different clinical phenotypes of the disease. In both cases the diagnosis was confirmed by genetic examinations that revealed homozygous mutations in the fibrinogen A genes. 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