Department of Internal Medicine, Beth Israel Medical Center, Albert Einstein College of Medicine, New York, NY 10003, USA.
Clin Lymphoma Myeloma Leuk. 2013 Aug;13(4):360-9. doi: 10.1016/j.clml.2013.03.011.
Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies. Among these, hepatosplenic γδ T-cell lymphoma (HTCL) represents an aggressive and treatment-resistant subgroup for which new avenues of treatment are critically needed. HTCL is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in hepatosplenomegaly and peripheral blood cytopenias. Another characteristic feature is the expression of γδ T-cell receptors. HTCL exhibits a rapid progressive course and an extremely poor response to currently known therapeutic strategies, with a 5-year overall survival rate of only 7%. In this review, we discuss the clinical, pathologic, and molecular characteristics of this disease, along with the challenges that are associated with its diagnosis and treatment.
外周 T 细胞淋巴瘤是一组异质性的淋巴恶性肿瘤。其中，肝脾 γδ T 细胞淋巴瘤（HTCL）是一种侵袭性和治疗抵抗性的亚组，迫切需要新的治疗途径。HTCL 的特征是恶性细胞的主要结外分布，伴有肝脏、脾脏和骨髓的典型窦内浸润，导致肝脾肿大和外周血细胞减少。另一个特征是 γδ T 细胞受体的表达。HTCL 表现出快速进展的病程和对目前已知治疗策略的极差反应，5 年总生存率仅为 7%。在这篇综述中，我们讨论了这种疾病的临床、病理和分子特征，以及与诊断和治疗相关的挑战。
