{"title":"严重的获得性因子 VII 缺乏症并发再生障碍性贫血,使用皮质类固醇治疗成功。","authors":"Batton Romain, Chamouni Pierre, Carrette Marion, Sauvêtre Gaëtan, Barbay Virginie","doi":"10.1097/MBC.0000000000001323","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2000,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids.\",\"authors\":\"Batton Romain, Chamouni Pierre, Carrette Marion, Sauvêtre Gaëtan, Barbay Virginie\",\"doi\":\"10.1097/MBC.0000000000001323\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":8992,\"journal\":{\"name\":\"Blood Coagulation & Fibrinolysis\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.2000,\"publicationDate\":\"2024-09-23\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Blood Coagulation & Fibrinolysis\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1097/MBC.0000000000001323\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Blood Coagulation & Fibrinolysis","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/MBC.0000000000001323","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
因子 VII 缺乏症是一种罕见的止血疾病,可导致严重的临床后果。由于报道的病例很少,这种疾病的治疗指南仍不明确。在本报告中,我们介绍了一例患有髓质发育不全的老年女性获得性因子 VII 缺乏症(aFVII)病例。最初的临床表现为血红蛋白症,并伴有凝血酶原时间的快速延长。医生及时采取了干预措施,补充营养以解决缺乏症问题。随后的实验室检测未能发现任何特异性 VII 因子抑制剂。考虑到患者体弱多病,医生仅对其进行了皮质类固醇免疫抑制治疗。潜在的诱发机制可能包括反复发作的败血症或潜在的自身免疫疾病。为了更好地了解 aFVII 缺乏症的病因和最佳治疗方法,有必要开展进一步的研究。
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.
期刊介绍:
Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components