Afibrinogenemia

Afibrinogenemia is a rare, severe hematologic disorder characterized by the complete absence or undetectable levels of circulating fibrinogen (coagulation factor I) in the blood Typically inherited in an autosomal recessive pattern due to mutations in the fibrinogen gene cluster (*FGA*, *FGB*, or *FGG*), it can also rarely be acquired secondary to severe hepatic failure or hyperconsumptive states Pathophysiologically, the absence of fibrinogen impairs both stable fibrin clot formation and normal...

Afibrinogenemia is a rare, severe hematologic disorder characterized by the complete absence or undetectable levels of circulating fibrinogen (coagulation factor I) in the blood Clinically, affected individuals experience a lifelong bleeding diathesis that frequently presents in the neonatal period with prolonged umbilical cord hemorrhage, followed by mucosal bleeding, hemarthroses, hematomas, and potentially fatal spontaneous intracranial hemorrhage, paradoxical thromboembolism, or recurrent ea...

Typically inherited in an autosomal recessive pattern due to mutations in the fibrinogen gene cluster (*FGA*, *FGB*, or *FGG*), it can also rarely be acquired secondary to severe hepatic failure or hyperconsumptive states

Clinically, affected individuals experience a lifelong bleeding diathesis that frequently presents in the neonatal period with prolonged umbilical cord hemorrhage, followed by mucosal bleeding, hemarthroses, hematomas, and potentially fatal spontaneous intracranial hemorrhage, paradoxical thromboembolism, or recurrent early pregnancy loss