Kasai Hajime, Terada Jiro, Hoshi Hiromasa, Urushibara Takashi, Kato Fumiaki, Nishimura Rintaro, Tatsumi Koichiro
Department of Respirology, Graduate School of Medicine, Chiba University, Japan.
Intern Med. 2017;56(4):425-428. doi: 10.2169/internalmedicine.56.7614. Epub 2017 Feb 15.
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication that occurs in association with various diseases including coagulation disorders. In rare cases, it is caused by hemophilia. A 48-year-old man was admitted to our hospital for a third time due to DAH. Although the cause of DAH could not be identified by bronchoscopy or laboratory tests, a good response to corticosteroids suggested idiopathic DAH with pulmonary capillaritis. The patient was diagnosed with hemophilia B based on the results of a detailed inquiry, a mildly prolonged activated partial thromboplastin time, and low factor IX activity. Hemophilia may be an underlying factor that exacerbates the bleeding of patients with DAH, even when they show a good response to corticosteroids.
弥漫性肺泡出血(DAH)是一种危及生命的并发症,与包括凝血障碍在内的多种疾病相关。在罕见情况下,它由血友病引起。一名48岁男性因DAH第三次入住我院。尽管通过支气管镜检查或实验室检查无法确定DAH的病因,但对皮质类固醇有良好反应提示为伴有肺毛细血管炎的特发性DAH。根据详细询问结果、活化部分凝血活酶时间轻度延长以及因子IX活性降低,该患者被诊断为B型血友病。血友病可能是加重DAH患者出血的潜在因素,即使他们对皮质类固醇表现出良好反应。