Adult T-cell leukemia/lymphoma (ATLL) is an aggressive hematologic malignancy linked to HTLV-1 infection, which is refractory to therapy. The precise mechanism of oncogenesis in ATLL is incompletely understood, however, oncogenic viral genes Tax and Hbz are implicated, and recent large genomic and transcriptome studies provide further insight. Despite progress in understanding the disease, survival and outcome with current therapies remain poor. Long-term survivors are reported, primarily among those with indolent disease or activating CC chemokine receptor 4 mutations, however, allogeneic hematopoietic stem cell transplant is the only curative treatment option. The majority of patients succumb to their disease and ongoing and collaborative research efforts are needed. I will review recent updates in HTLV-1-associated ATLL epidemiology, pathogenesis, therapy, and prevention.

成人 T 细胞白血病/淋巴瘤 (ATLL) 是一种与 HTLV-1 感染相关的侵袭性血液恶性肿瘤，难以治疗。ATLL 中肿瘤发生的确切机制尚不完全清楚，然而，致癌病毒基因 Tax 和 Hbz 参与其中，最近的大型基因组和转录组研究提供了进一步的见解。尽管在了解该疾病方面取得了进展，但当前疗法的存活率和结果仍然很差。据报道，长期幸存者主要是那些患有惰性疾病或激活 CC 趋化因子受体 4 突变的患者，然而，同种异体造血干细胞移植是唯一的治愈性治疗选择。大多数患者死于他们的疾病，需要持续的合作研究工作。我将回顾 HTLV-1 相关 ATLL 流行病学的最新更新，