Department of Clinical Medicine and Surgery, Gastroenterology, University of Naples "Federico II", Via S. Pansini n° 5, 80131, Naples, Italy.
Department of Hematology, University of Naples "Federico II", Via S. Pansini n° 5, 80131, Naples, Italy.
Dig Dis Sci. 2021 Feb;66(2):408-411. doi: 10.1007/s10620-020-06670-z. Epub 2020 Oct 22.
Hepatitis-associated aplastic anemia is a well-recognized clinical syndrome in which marrow failure follows the development of hepatitis. Although aplastic anemia is intimately related to paroxysmal nocturnal hemoglobinuria, until now, no cases of PNH-associated hepatitis have been described. We report a case of recurrent acute hepatitis preceding the clinical onset of PNH. Treatment of PNH with the complement inhibitor eculizumab (Soliris) prevented both recurrences of episodes of intravascular hemolysis and liver enzyme alteration. This is the first known published case of PNH-associated hepatitis.
肝炎相关性再生障碍性贫血是一种公认的临床综合征,其骨髓衰竭发生在肝炎之后。尽管再生障碍性贫血与阵发性睡眠性血红蛋白尿密切相关,但到目前为止,尚未有与阵发性睡眠性血红蛋白尿相关的肝炎的病例报道。我们报告了一例在阵发性睡眠性血红蛋白尿临床发病前反复发作急性肝炎的病例。用补体抑制剂依库珠单抗(Soliris)治疗阵发性睡眠性血红蛋白尿可预防血管内溶血和肝酶改变的再次发作。这是首例已知的与阵发性睡眠性血红蛋白尿相关的肝炎的病例。