Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis · 2024
Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids.
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Batton Romain, Pierre Chamouni, Carrette Marion, Gaëtan Sauvêtre, Barbay Virginie
Аннотация
Factor VII deficiency is a rare hemostatic disorder that can lead to severe clinical outcomes. Due to the scarcity of reported cases, treatment guidelines for this condition remain unclear. In this report, we present a case of acquired factor VII deficiency (aFVII) in an elderly female with medullary aplasia. The initial clinical manifestation was hemarthrosis, accompanied by a rapid increase in prothrombin time. Prompt intervention involved supplementation to address the deficiency. Subsequent laboratory testing failed to detect any specific factor VII inhibitor. Considering the patient's frailty, immunosuppressive therapy comprising only corticosteroids was administered. The potential triggering mechanisms may include recurrent episodes of sepsis or an underlying autoimmune condition. Further research is necessary to better understand the etiology and optimal management of aFVII deficiency.