Iseda Norifumi, Yoshizumi Tomoharu, Toshima Takeo, Morinaga Akinari, Tomiyama Takahiro, Takahashi Junichi, Motomura Takashi, Mano Yohei, Itoh Shinji, Harada Noboru, Ikegami Toru, Soejima Yuji
Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Surg Case Rep. 2018 Aug 29;4(1):101. doi: 10.1186/s40792-018-0505-5.
Hemophagocytic syndrome (HPS) is a rare and potentially fatal complication following liver transplantation.
A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus infection underwent living donor liver transplantation using the right posterior section of her husband's liver (graft volume, 581 g; 56.8% of the recipient's standard liver volume). She developed small-for-size syndrome on postoperative day (POD) 7, and HPS was diagnosed on POD 12 by bone marrow aspiration (white blood cells, 300/μL; neutrophils, 30/μL). Given that she tested negative for viral (hepatitis B virus and cytomegalovirus) and bacterial infections, it was considered likely to be secondary HPS. Steroid pulse therapy was initiated, and her white blood cell count increased to 4290/μL on POD 15, indicating that her peripheral blood leukocytes had improved. There were no surgical complications, but the patient died of prolonged graft dysfunction with bacterial sepsis on POD 14.
We report a rare case of HPS occurring 2 weeks after living donor liver transplantation with a right posterior section graft, diagnosed early via bone marrow aspiration. This clinical course implies an association between HPS and graft dysfunction such as small-for-size syndrome. Further studies of the mechanism of hypercytokinemia-induced HPS are required to confirm the optimal treatment for HPS.
噬血细胞综合征（HPS）是肝移植后一种罕见且可能致命的并发症。
一名63岁女性，因乙型肝炎病毒感染导致失代偿期肝硬化，接受了活体肝移植，供肝为其丈夫肝脏的右后叶（移植肝体积581g；占受者标准肝体积的56.8%）。术后第7天她出现了小肝综合征，术后第12天通过骨髓穿刺确诊为HPS（白细胞计数300/μL；中性粒细胞计数30/μL）。鉴于她的病毒（乙型肝炎病毒和巨细胞病毒）及细菌感染检测均为阴性，考虑可能为继发性HPS。开始给予类固醇冲击治疗，术后第15天她的白细胞计数升至4290/μL，提示外周血白细胞情况有所改善。未出现手术并发症，但患者于术后第14天因移植肝功能长期不良合并细菌败血症死亡。
我们报告了一例罕见的活体肝移植后右后叶移植肝2周后发生HPS的病例，通过骨髓穿刺早期确诊。该临床过程提示HPS与小肝综合征等移植肝功能障碍之间存在关联。需要进一步研究高细胞因子血症诱导的HPS的机制，以确定HPS的最佳治疗方法。
