Taniai Nobuhiko, Akimaru Koho, Kawano Youichi, Mizuguchi Yoshiaki, Shimizu Tetuya, Takahashi Tsubasa, Mamada Yasuhiro, Yoshida Hiroshi, Tajiri Takashi
First Department of Surgery, Nippon Medical School, Tokyo, Japan.
Hepatogastroenterology. 2005 May-Jun;52(63):923-6.
Hemophagocytic syndrome (HPS) is a hypercytokinemia caused by activated T lymphocytes and macrophages in immunologically compromised patients. We report a 37-year-old female who was diagnosed with HPS after undergoing living-donor liver transplantation (LDLT) for fulminant liver failure of unknown etiology. After liver transplantation, recipients with pancytopenia should be tested for serum ferritin. When the serum ferritin is abnormal, the bone marrow should be biopsied to screen for HPS as soon as possible. If the condition is caught early and promptly treated, the outcome of this devastating condition might be improved. In addition, HPS should be ruled out in LDLT candidates with acute liver failure before their operations.
噬血细胞综合征（HPS）是一种由免疫功能低下患者体内活化的T淋巴细胞和巨噬细胞引起的高细胞因子血症。我们报告一例37岁女性，因病因不明的暴发性肝衰竭接受活体肝移植（LDLT）后被诊断为HPS。肝移植后，全血细胞减少的受者应检测血清铁蛋白。当血清铁蛋白异常时，应尽快进行骨髓活检以筛查HPS。如果病情能早期发现并及时治疗，这种严重疾病的预后可能会得到改善。此外，对于急性肝衰竭的LDLT候选者，术前应排除HPS。
