T细胞原淋巴细胞白血病（T-PLL）是成熟T细胞的罕见且侵袭性的肿瘤。大多数T-PLL患者表现为淋巴细胞增多症、贫血、血小板减少和肝脾肿大。正确识别T-PLL至关重要，因为该疾病的治疗与其他T细胞肿瘤不同。2019年，T-PLL国际研究组（TPLL-ISG）制定了T-PLL的诊断、分期和治疗反应评估标准，旨在协调研究工作并支持临床决策。T-PLL的病因通常由T细胞白血病1（TCL1）过度表达和ATM丧失驱动，这些遗传变异被纳入了TPLL-ISG的诊断标准。TCL1家族成员和ATM之间的相互作用似乎在T细胞肿瘤谱中是独特的。T细胞受体、其下游激酶和JAK/STAT信号转导通路在疾病发病机制中的作用也是新兴的主题，并且具有明显的治疗意义。尽管对疾病发病机制的理解有所改善，但阿雷木松仍然是治疗具有治疗指征的初始疗法，因为它具有较高的治疗反应率。不幸的是，所取得的治疗反应很少持久，并且大多数患者不适合进行造血干细胞移植巩固治疗。对T-PLL发病机制的进一步了解揭示了可能改变这种淋巴增殖性肿瘤的自然历史的新型治疗易感性，这将是本简明综述的重点。

T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm of mature T-cells. Most patients with T-PLL present with lymphocytosis, anemia, thrombocytopenia, and hepatosplenomegaly. Correct identification of T-PLL is essential because treatment for this disease is distinct from that of other T-cell neoplasms. In 2019, the T-PLL International Study Group (TPLL-ISG) established criteria for the diagnosis, staging, and assessment of response to treatment of T-PLL with the goal of harmonizing research efforts and supporting clinical decision-making. T-PLL pathogenesis is commonly driven by T-cell leukemia 1 (TCL1) overexpression and ATM loss, genetic alterations that are incorporated into the TPLL-ISG diagnostic criteria. The cooperativity between TCL1 family members and ATM is seemingly unique to T-PLL across the spectrum of T-cell neoplasms. The role of the T-cell receptor, its downstream kinases, and JAK/STAT signaling are also emerging themes in disease pathogenesis and have obvious therapeutic implications. Despite improved understanding of disease pathogenesis, alemtuzumab remains the frontline therapy in the treatment of naïve patients with indications for treatment given its high response rate. Unfortunately, the responses achieved are rarely durable, and the majority of patients are not candidates for consolidation with hematopoietic stem cell transplantation. Improved understanding of T-PLL pathogenesis has unveiled novel therapeutic vulnerabilities that may change the natural history of this lymphoproliferative neoplasm and will be the focus of this concise review.