Department of Surgery, Ajou University School of Medicine, Suwon, Korea.
Department of Pathology, Ajou University School of Medicine, Suwon, Korea.
Yonsei Med J. 2021 Mar;62(3):274-277. doi: 10.3349/ymj.2021.62.3.274.
Hemophagocytic syndrome (HPS) is a rare but potentially life-threatening disease in kidney transplant recipients, and is caused by systemic proliferation of macrophages actively phagocytizing other blood cells in the bone marrow, lymph nodes, and the spleen. Here, we report a 40-year-old male kidney transplant recipient who presented with fever, bicytopenia, and elevated liver enzymes 2 months after transplantation. Given that cytomegalovirus antigenemia and real-time polymerase chain reaction tests were positive, liver biopsy was performed under an assumption of cytomegalovirus-induced hepatitis. Hepatic histology revealed multifocal microabscess with cytomegalovirus inclusion bodies, marked Kupffer cell hyperplasia, and erythrophagocytosis by activated macrophages. As laboratory findings such as hyperferritinemia, elevated serum lactate dehydrogenase, low natural killer cell activity, and high soluble interleukin-2 receptor were also compatible with HPS, the recipient was diagnosed as having cytomegalovirus-induced hepatitis combined with reactive HPS. Following intravenous ganciclovir therapy with continuous administration of tacrolimus and corticosteroid, the symptoms resolved and laboratory findings were normalized. As far as we know, this is the first report of cytomegalovirus-induced hepatitis combined with reactive HPS in a kidney transplant recipient that is diagnosed by liver biopsy.
噬血细胞综合征(HPS)是肾移植受者中一种罕见但潜在危及生命的疾病,是由骨髓、淋巴结和脾脏中的巨噬细胞系统性增殖引起的,这些巨噬细胞主动吞噬其他血细胞。在这里,我们报告了一例 40 岁男性肾移植受者,他在移植后 2 个月出现发热、两系血细胞减少和肝酶升高。鉴于巨细胞病毒抗原血症和实时聚合酶链反应检测呈阳性,假设为巨细胞病毒诱导的肝炎进行了肝活检。肝组织学显示伴有巨细胞病毒包涵体的多灶性微脓肿,明显的枯否细胞增生和活化巨噬细胞的红细胞吞噬作用。由于高血清铁蛋白血症、血清乳酸脱氢酶升高、自然杀伤细胞活性降低和可溶性白细胞介素-2 受体升高的实验室发现也与 HPS 相符,受者被诊断为巨细胞病毒诱导的肝炎合并反应性 HPS。静脉注射更昔洛韦联合持续给予他克莫司和皮质类固醇治疗后,症状缓解,实验室检查结果恢复正常。据我们所知,这是首例通过肝活检诊断的肾移植受者巨细胞病毒诱导的肝炎合并反应性 HPS。