Seo Ji Young, Seo Dong Dae, Jeon Tae Joo, Oh Tae Hoon, Shin Won Chang, Choi Won Choong, Yoo Soo Jin, Han Tae Hee
Department of Internal Medicine, Inje University College of Medicine, Sanggye Paik Hospital, Seoul, Korea.
Korean J Hepatol. 2010 Mar;16(1):79-82. doi: 10.3350/kjhep.2010.16.1.79.
Hemophagocytic syndrome (HPS) is a rare but serious condition that is histopathologically characterized by activation of macrophage or histiocytes with hemophagocytosis in bone marrow and reticuloendothelial systems. Clinically it presents with high fever, hepatosplenomegaly, pancytopenia, liver dysfunction, and hyperferritinemia. Hepatitis A virus is a very rare cause of secondary HPS. We report a case of a 22-year-old woman infected by hepatitis A virus who was consequently complicated with HPS. She presented typical clinical features of acute hepatitis A, and showed clinical and biochemical improvements. However, HPS developed as a complication of acute hepatitis A and the patient died of intraperitoneal bleeding caused by hepatic decompensation and disseminated intravascular coagulation.
噬血细胞综合征（HPS）是一种罕见但严重的病症，其组织病理学特征为骨髓和网状内皮系统中巨噬细胞或组织细胞活化并伴有噬血细胞现象。临床上表现为高热、肝脾肿大、全血细胞减少、肝功能障碍和高铁蛋白血症。甲型肝炎病毒是继发性HPS非常罕见的病因。我们报告一例22岁感染甲型肝炎病毒的女性患者，该患者继而并发HPS。她表现出急性甲型肝炎的典型临床特征，并在临床和生化方面有所改善。然而，HPS作为急性甲型肝炎的并发症出现，患者因肝衰竭和弥散性血管内凝血导致的腹腔内出血而死亡。
