肝脾T细胞淋巴瘤是一种罕见的T细胞淋巴瘤，主要由表达γ/δ T细胞受体的淋巴细胞的细胞毒性T细胞的肿瘤性增殖引起。 7q 等染色体和 8 三体是与肝脾 T 细胞淋巴瘤相关的最常见的染色体异常，大多数患者存在与染色质重塑或 JAK/STAT 系统相关的基因突变。肝脾 T 细胞淋巴瘤可以模仿各种传染病、免疫状况和其他恶性肿瘤。患者通常表现为非特异性全身症状、脾脏和肝脏肿大，并伴有不同程度的血细胞减少。这种疾病非常罕见，加上淋巴瘤通常不涉及淋巴结受累，导致诊断非常困难，这不可避免地延迟了治疗的开始。由于这种淋巴瘤的晚期表现和侵袭性，治疗这种淋巴瘤非常困难，经常导致其临床病程快速进展并且对常规化疗无效。缺乏针对其治疗的国际指南，并且在大多数情况下，治疗以病例系列为指导。在这里，我们重点介绍单个血液学转诊中心 10 年来肝脾 T 细胞淋巴瘤的临床病理特征和治疗，并回顾文献。© 2023 作者。由巴塞尔 S. Karger AG 出版。

Hepatosplenic T-cell lymphoma is a rare form of T-cell lymphoma that predominantly emerges from neoplastic proliferation of cytotoxic T cells of γ/δ T-cell receptor-expressing lymphocytes. Isochromosome 7q and trisomy 8 are the most prevalent chromosomal abnormalities associated with hepatosplenic T-cell lymphoma, and most patients have mutations in genes related to chromatin remodeling or the JAK/STAT system. Hepatosplenic T-cell lymphoma can mimic various infectious diseases, immunological conditions, and other malignancies. Patients usually present with nonspecific constitutional symptoms and spleen and liver enlargement, with variable degrees of cytopenia. The rarity of this disease, coupled with the lack of lymph node involvement that is usually seen in lymphomas, causes significant difficulty in diagnosis, which inevitably delays the initiation of treatment. Managing this lymphoma is arduous because of its late presentation and aggressive nature, frequently resulting in rapid progression in its clinical course and refractoriness to conventional chemotherapy. There is a lack of international guidelines for its treatment, and in most cases, treatment is guided by case series. Here, we highlight the clinicopathological features and management of hepatosplenic T-cell lymphoma over a 10-year span in a single hematology referral center and review the literature.© 2023 The Author(s). Published by S. Karger AG, Basel.