Digestive and Lifestyle Diseases, Department of Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8544, Japan.
Department of Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, 8‑35‑1 Sakuragaoka, Kagoshima, 890‑8544, Japan.
Clin J Gastroenterol. 2023 Jun;16(3):416-421. doi: 10.1007/s12328-023-01771-4. Epub 2023 Feb 22.
An 87-year-old man consulted a former doctor with a complaint of black stool and was admitted to hospital because of anemia and multiple gastric ulcers. The laboratory findings showed that his hepatobiliary enzyme levels and inflammatory response were elevated. Computed tomography showed hepatosplenomegaly and enlarged intra-abdominal lymph nodes. Two days later, he was transferred to our hospital due to deterioration of his liver function. Since he had low level of consciousness and his ammonia level was high, we diagnosed him with acute liver failure (ALF) with hepatic coma, and started on-line hemodiafiltration. As the cause of ALF, we suspected hepatic involvement of a hematologic tumor because of high lactate dehydrogenase and soluble interleukin-2 receptor levels and large abnormal lymphocyte-like cells in the peripheral blood. Because of his poor general condition, bone marrow and other histological examinations were difficult, and he died on the third day of hospitalization. Pathological autopsy showed marked hepatosplenomegaly and the proliferation of large abnormal lymphocyte-like cells in the bone marrow, liver, spleen, and lymph nodes. Immunostaining revealed aggressive natural killer-cell leukemia (ANKL).We herein report a rare case of the development of ALF with coma due to ANKL with a review of the relevant literature.
一位 87 岁男性因黑便和贫血以及多发性胃溃疡就诊于前医生,并因贫血和多发性胃溃疡入院。实验室检查发现其肝胆酶水平和炎症反应升高。计算机断层扫描显示肝脾肿大和腹腔内淋巴结肿大。两天后,因肝功能恶化转入我院。由于患者意识水平低,血氨水平高,我们诊断为肝性脑病急性肝衰竭(ALF),并开始在线血液透析滤过。由于 ALF 的原因,我们怀疑血液系统肿瘤累及肝脏,因为乳酸脱氢酶和可溶性白细胞介素-2 受体水平高,外周血中有大量异常淋巴细胞样细胞。由于患者一般情况较差,骨髓等组织学检查困难,入院后第三天死亡。病理尸检显示肝脾肿大明显,骨髓、肝脏、脾脏和淋巴结内有大量异常淋巴细胞样细胞增生。免疫组化显示侵袭性自然杀伤细胞白血病(ANKL)。本文报告一例罕见的因 ANKL 导致昏迷的 ALF 病例,并复习相关文献。