Division of Medical Oncology and Hematology, Department of Internal Medicine, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea.
Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81, Irwon-ro, Gangnam-gu, Seoul, 06351, Korea.
Ann Hematol. 2023 Jul;102(7):1867-1877. doi: 10.1007/s00277-023-05182-w. Epub 2023 May 16.
Hepatosplenic T cell lymphoma (HSTCL) is a rare and aggressive lymphoma with no standard treatment and poor treatment response. From 2001-2021, 20 from a lymphoma cohort of 7247 patients (0.27%) were diagnosed with HSTCL at Samsung Medical Center. The median age at the time of diagnosis was 37.5 (range, 17-72) years, and 75.0% of patients were male. Most patients had B symptoms, hepatomegaly, and splenomegaly. Lymphadenopathy was found in only 31.6% of patients, and increased PET-CT uptake was found in 21.1% of patients. Thirteen patients (68.4%) expressed T cell receptor (TCR) γδ, and 6 patients (31.6%) expressed TCRαβ. The median progression-free survival (PFS) for the entire cohort was 7.2 months (95% CI, 2.9-12.8), and the median overall survival (OS) was 25.7 months (95% CI, not calculated). In subgroup analysis, the overall response rate (ORR) was 100.0% in the ICE/Dexa group and 53.8% in the anthracycline-based group, and the complete response rate was 83.3% in the ICE/Dexa group and 38.5% in the anthracycline-based group. The ORR was 50.0% in the TCRαβ group and 83.3% in the TCRγδ group. The OS was not reached in the autologous hematopoietic stem cell transplantation (HSCT) group and was 16.0 months (95% CI, 15.1-16.9) in the non-transplant group at the data cutoff time (P value 0.015). In conclusion, HSTCL is rare but has a very poor prognosis. The optimal treatment strategy is not defined. More genetic and biological information is needed.
肝脾 T 细胞淋巴瘤（HSTCL）是一种罕见且侵袭性强的淋巴瘤，目前尚无标准治疗方法，且治疗反应较差。2001 年至 2021 年期间，在三星医疗中心的 7247 例淋巴瘤患者队列中，有 20 例（0.27%）被诊断为 HSTCL。诊断时的中位年龄为 37.5 岁（范围，17-72 岁），75.0%的患者为男性。大多数患者有 B 症状、肝肿大和脾肿大。仅 31.6%的患者有淋巴结肿大，21.1%的患者 PET-CT 摄取增加。13 例（68.4%）患者表达 T 细胞受体（TCR）γδ，6 例（31.6%）患者表达 TCRαβ。整个队列的中位无进展生存期（PFS）为 7.2 个月（95%CI，2.9-12.8），中位总生存期（OS）为 25.7 个月（95%CI，未计算）。在亚组分析中，ICE/Dexa 组的总缓解率（ORR）为 100.0%，蒽环类药物为 53.8%，ICE/Dexa 组完全缓解率为 83.3%，蒽环类药物为 38.5%。TCRαβ 组的 ORR 为 50.0%，TCRγδ 组为 83.3%。自体造血干细胞移植（HSCT）组的 OS 未达到，截止数据时，非移植组的 OS 为 16.0 个月（95%CI，15.1-16.9）（P 值=0.015）。总之，HSTCL 较为罕见，但预后极差。目前尚未确定最佳的治疗策略，需要更多的遗传和生物学信息。
