Acquired, non-amyloid related factor X deficiency: review of the literature.

摘要: Summary.  Factor X (FX) deficiency is a rare coagulopathy due to congenital (Stuart–Prower disease) or in association with primary amyloidosis. Acquired and isolated FX occurring the absence of plasma cell dyscrasia has only been infrequently described. After recently diagnosing treating case acquired, deficiency, we embarked upon review literature help guide clinicians who may face this clinical situation. The was reviewed identify cases isolated, acquired unrelated use vitamin K antagonists, There were 34 identified, malignancy, drug exposure infection. majority described (38%) preceded by non-specific respiratory viral illness. initial presentation variable, ranging from no bleeding life-threatening haemorrhage. Twenty per cent patients had musculoskeletal resembling haemophilia. Both prothrombin time activated partial thromboplastin markedly prolonged nearly all patients. In 26% patients, specific inhibitor identified. Numerous therapies have utilized including high-dose glucocorticoids, exchange fresh frozen intravenous immunoglobulin. 18% resolved spontaneously. All achieved complete recovery. factor disorder, commonly associated preceding illness circulating inhibitor. Although multiple treatment modalities variable success, many cases, it self-limited condition.