Department of Gastroenterology and Hepatology, Tenri Hospital, 200 Mishima-cho, Tenri, Nara, 632-8552, Japan.
Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Clin J Gastroenterol. 2023 Oct;16(5):698-701. doi: 10.1007/s12328-023-01813-x. Epub 2023 May 11.
A man in his 60s was admitted because of abdominal pain and fatigue. Contrast enhanced computed tomography (CECT) showed a hypovascular tumor, 7 cm in size, in the left lobe of liver. He had no history of alcohol consumption. HBs antigen and HCV antibody were negative. For definitive diagnosis, biopsy of the hepatic tumor was performed. After the biopsy, the patient suddenly got high fever, and blood tests showed WBC 22,000/L, Hb 8.9 g/dL, Plts 11.6 × 10/L, AST 140 IU/L, ALT 93 IU/L, LDH 635 U/L. He died on the following day despite of supportive therapy. Autopsy revealed that the hepatic tumor was poorly differentiated hepatocellular carcinoma (HCC) and that hemophagocytic macrophages were found in the bone marrow and spleen. Based on the pathological findings of autopsy, he was finally diagnosed with hemophagocytic lymphohistiocytosis (HLH) associated with HCC. HLH is a rare and life-threaded disorder of immune overactivation. Malignancy-associated HLH is well-known; however, it is usually associated with malignant lymphoma. To our knowledge, this is the first reported case of HLH associated with HCC, which was diagnosed by autopsy. Although extremely rare, our case highlights that HLH should be considered as a differential diagnosis of unknown high fever and bicytopenia in patients with solid tumors, including HCC.
一位 60 多岁的男性因腹痛和乏力入院。增强 CT 显示肝左叶有一个 7cm 大的低血供肿瘤。他没有饮酒史。HBs 抗原和 HCV 抗体均为阴性。为明确诊断，对肝肿瘤进行了活检。活检后，患者突然高热，血液检查显示白细胞 22,000/L，血红蛋白 8.9 g/dL，血小板 11.6×10/L，AST 140 IU/L，ALT 93 IU/L，LDH 635 U/L。尽管进行了支持治疗，他还是在第二天死亡。尸检显示肝肿瘤为低分化肝细胞癌（HCC），骨髓和脾脏中发现噬血细胞巨噬细胞。根据尸检的病理发现，他最终被诊断为与 HCC 相关的噬血细胞性淋巴组织细胞增生症（HLH）。HLH 是一种罕见的免疫过度激活的危及生命的疾病。恶性肿瘤相关的 HLH 是众所周知的，但它通常与恶性淋巴瘤相关。据我们所知，这是首例通过尸检诊断的与 HCC 相关的 HLH 病例。虽然极其罕见，但我们的病例强调了 HLH 应被视为包括 HCC 在内的实体瘤患者不明原因高热和两系血细胞减少的鉴别诊断。
