Yamaguchi Tomoya, Kudo Naoko, Endo Susumu, Usui Takeo, Imashuku Shinsaku
Department of Internal Medicine, Uji-Tokushukai Medical Center, Uji 611-0042, Japan.
Division of Hematology, Takasago-Seibu Hospital, Takasago 676-0812, Japan.
Case Rep Hematol. 2018 Nov 13;2018:6757345. doi: 10.1155/2018/6757345. eCollection 2018.
This report describes six elderly patients with acquired hemophilia A (AHA), including four individuals aged ≥90 years. Bleeding symptoms were subcutaneous or intramuscular hemorrhage (=4), hematuria (=1), and hemorrhagic shock after tooth extraction (=1). Factor VIII (FVIII) activity ranged from <1.0% to 3.0%, and anti-FVIII inhibitor titers ranged from 8.8 to 240 BU/mL. Treatment was administered at the discretion of the responsible physician. Hemostatic agents applied in the six patients comprised rFVIIa (NovoSeven®) (=4), APCC (Feiba®) (=2), and fresh frozen plasma/plasma exchange (=1). Agents employed for inhibitor eradication comprised prednisolone only (=3), prednisolone with cyclophosphamide (=1), prednisolone with cyclosporine (=1), and prednisolone with rituximab (=1). In five patients, management was successful, with complete response. Treatment failed in the patient with the highest inhibitor level (240 BU/mL) in whom treatment with APCC (Feiba®; 100 U/kg/dose, three doses) and prednisolone (0.5 mg/kg/day) was followed by several episodes of relapse. The present data demonstrate that AHA severity shows wide variation in elderly subjects, indicating the necessity of individualized management.
本报告描述了6例获得性血友病A(AHA)老年患者,其中包括4例年龄≥90岁的患者。出血症状包括皮下或肌肉内出血(4例)、血尿(1例)以及拔牙后出血性休克(1例)。凝血因子VIII(FVIII)活性范围为<1.0%至3.0%,抗FVIII抑制剂滴度范围为8.8至240 BU/mL。治疗由负责医生酌情进行。6例患者应用的止血剂包括重组活化凝血因子VII(rFVIIa,诺其®)(4例)、活化凝血酶原复合物(APCC,非巴®)(2例)以及新鲜冰冻血浆/血浆置换(1例)。用于消除抑制剂的药物仅包括泼尼松龙(3例)、泼尼松龙联合环磷酰胺(1例)、泼尼松龙联合环孢素(1例)以及泼尼松龙联合利妥昔单抗(1例)。5例患者治疗成功,获得完全缓解。抑制剂水平最高(240 BU/mL)的患者治疗失败,该患者接受APCC(非巴®;100 U/kg/剂量,3剂)和泼尼松龙(0.5 mg/kg/天)治疗后出现多次复发。目前的数据表明,AHA严重程度在老年患者中差异很大,这表明有必要进行个体化管理。