Okoshi Yasushi, Akiyama Hideki, Inoue Takashi, Koyama Takatoshi, Kono Natsu, Matsumura Tomoko, Mizuchi Daisuke, Mori Shin-ichiro, Ohashi Kazuteru, Sakamaki Hisashi
Hematology Division, Tokyo Metropolitan Komagome Hospital.
Rinsho Ketsueki. 2003 Jan;44(1):14-8.
A rare case of a long-standing idiopathic acquired blood coagulation factor V (FV) inhibitor is reported. A 78 year-old male was admitted complaining of hematuria and tarry stools of two weeks' duration. He was noted to have a prolonged prothrombin time and activated partial thromboplastin time. A mixing study suggested the existence of an inhibitor and the activity of FV was less than 1%. Western blot analysis revealed the presence of anti-FV antibodies in the patient's serum. Because of significant bleeding tendencies, the patient received fresh-frozen plasma, platelet transfusions and methylprednisolone. His PT, APTT and FV activity were partially corrected although the disease was exacerbated when the steroid dose was tapered off. The patient was then treated with low dose cyclophosphamide. The majority of the cases with acquired FV inhibitor occur spontaneously in older patients but this condition may be associated with a surgical procedure, administration of antibiotics, tuberculosis or malignant diseases; some of them are, however, idiopathic. No underlying disease was detected in our present patient. The inhibitor disappears within 10 weeks in most patients and the prolonged presence of an idiopathic FV inhibitor is rare.
报告了一例长期存在的特发性获得性凝血因子V(FV)抑制剂的罕见病例。一名78岁男性因持续两周的血尿和柏油样便入院。他的凝血酶原时间和活化部分凝血活酶时间延长。混合试验提示存在抑制剂,FV活性低于1%。Western印迹分析显示患者血清中存在抗FV抗体。由于明显的出血倾向,患者接受了新鲜冰冻血浆、血小板输注和甲泼尼龙治疗。尽管当类固醇剂量逐渐减小时病情加重,但他的PT、APTT和FV活性得到了部分纠正。然后患者接受了低剂量环磷酰胺治疗。大多数获得性FV抑制剂病例在老年患者中自发出现,但这种情况可能与外科手术、抗生素使用、结核病或恶性疾病有关;然而,其中一些是特发性的。在我们目前的患者中未检测到潜在疾病。大多数患者的抑制剂在10周内消失,特发性FV抑制剂长期存在的情况很少见。