학술논문

Clinical features of five cases of acquired factor V deficiency / 後天性凝固第V因子欠乏症5例の臨床的検討
Document Type
Journal Article
Source
臨床血液 / Rinsho Ketsueki. 2022, 63(7):733
Subject
Acquired factor V deficiency
Cross-mixing test
Inhibitor
Language
Japanese
ISSN
0485-1439
1882-0824
Abstract
Acquired factor V deficiency is a rare disease that presents with various bleeding symptoms because of the acquired production of factor V inhibitors and decrease in factor V activity. We have experienced five cases of acquired factor V deficiency diagnosed on the basis of abnormalities in coagulation tests in the last 10 years. All five patients were older men, of whom one had no bleeding symptoms, and three had a history of renal failure and malignant tumors. In the cross-mixing test, two of three cases demonstrated an inhibitor pattern, but one case showed a deficient pattern. In all cases, steroid treatment improved factor V activity as well as prothrombin time and activated partial thromboplastin time. However, patients with intracranial hemorrhage had a poor prognosis. Although this disease is rare, careful management is necessary, especially in the absence of bleeding symptoms and where cross-mixing test does not show an inhibitor pattern.

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