Božić Ozretić Dorotea, Piplović Vuković Tonka, Vuković Jonatan, Madunić Sanja, Podrug Kristian, Puljiz Željko
Department of Gastroenterology and Hepatology, University Hospital of Split, Split, Croatia.
University of Split, School of Medicine, Split, Croatia.
Case Rep Gastroenterol. 2020 Jul 28;14(2):383-390. doi: 10.1159/000508438. eCollection 2020 May-Aug.
Hepatitis-associated aplastic anemia is a rare syndrome in which bone marrow failure occurs within weeks to 1 year after attack of acute hepatitis. Studies suggest that cytotoxic T lymphocytes play a central role in bone marrow destruction, but the exact etiology remains unknown. Bone marrow transplantation or immunosuppressive therapy are primary curative options. We present a case of a young male who was admitted to the Department of Gastroenterology and Hepatology for acute hepatitis of an unknown cause. Liver biopsy revealed extensive inflammatory process with hepatocyte necrosis. Forty days later, new onset pancytopenia was identified. Bone marrow biopsy showed severe hypocellularity, and he was diagnosed with severe hepatitis-associated aplastic anemia. Treatment with cyclosporine was initiated, but with inadequate response, and pretransplant evaluation was started. Due to severe neutropenia, following alveotomy procedure, the patient developed deep neck infection with consequent airway obstruction. Despite urgent treatment, his condition deteriorated to sepsis with lethal outcome.
肝炎相关性再生障碍性贫血是一种罕见综合征,急性肝炎发作后数周内至1年内会发生骨髓衰竭。研究表明,细胞毒性T淋巴细胞在骨髓破坏中起核心作用,但确切病因仍不清楚。骨髓移植或免疫抑制治疗是主要的治愈选择。我们报告一例年轻男性病例,该患者因不明原因的急性肝炎入住胃肠病学和肝病科。肝脏活检显示广泛的炎症过程伴肝细胞坏死。40天后,发现新发全血细胞减少。骨髓活检显示严重细胞减少,他被诊断为严重肝炎相关性再生障碍性贫血。开始用环孢素治疗,但反应不佳,于是开始进行移植前评估。由于严重中性粒细胞减少,牙槽骨切开术后,患者发生深部颈部感染,继而出现气道梗阻。尽管进行了紧急治疗,但其病情恶化为败血症,最终死亡。