Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids

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.

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Publication Details

Journal
Blood Coagulation & Fibrinolysis
Published
2024-09-20
DOI
https://doi.org/10.1097/mbc.0000000000001323
Primary Topic
Hemophilia Treatment and Research
Type
article
Field-Weighted Citation Impact
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article

Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids

Benoit Romain, Virginie Barbay, Cassandra Pierre, Chelsea Marion et al.
Blood Coagulation & Fibrinolysis
Hemophilia Treatment and Research
article

Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids

Benoit Romain, Virginie Barbay, Cassandra Pierre, Chelsea Marion, Sauvêtre Gaëtan
article en

Abstract

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.

Blood Coagulation & FibrinolysisVol. 35(7)
Haemophilia Foundation Australia (AU), Centre Hospitalier Universitaire de Rouen (FR), Université de Rouen Normandie (FR)
Good health and well-being
Openalex Percentile: Top 71%
Hemophilia Treatment and Research
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Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids — Benoit Romain, Virginie Barbay, et al. · Blood Coagulation & Fibrinolysis (2024) | TGRS Research Map | TGRS