Bazarbachi Ali, Ghez David, Lepelletier Yves, Nasr Rihab, de Thé Hugues, El-Sabban Marwan E, Hermine Olivier
Department of Internal Medicine, American University of Beirut, Lebanon.
Lancet Oncol. 2004 Nov;5(11):664-72. doi: 10.1016/S1470-2045(04)01608-0.
Adult T-cell leukaemia or lymphoma is an aggressive malignant disease of mature activated T cells caused by human T-cell lymphotropic virus type I. Patients with this disease have a very poor outlook because of intrinsic chemoresistance and severe immunosuppression. In acute adult T-cell leukaemia, clinical trials in Japan show that although non-targeted combinations of chemotherapy improve response, they do not have a significant effect on complete remission and survival. Antiretroviral therapy with combination zidovudine and interferon alfa, which induces a high rate of complete remission and lengthens survival, should be the first treatment option in acute adult T-cell leukaemia. Patients with adult T-cell lymphoma might benefit from initial aggressive chemotherapy followed by antiretroviral therapy. To prevent relapse in all patients allogeneic bone-marrow transplantation when feasible, or additional targeted therapy, should be mandatory. Based on current pathophysiology, we discuss promising new drugs such as arsenic trioxide, proteasome inhibitors, retinoids, and angiogenesis inhibitors, as well as cellular immunotherapy.
成人T细胞白血病或淋巴瘤是一种由I型人类嗜T细胞病毒引起的成熟活化T细胞侵袭性恶性疾病。由于内在的化疗耐药性和严重的免疫抑制，该疾病患者的预后非常差。在急性成人T细胞白血病中，日本的临床试验表明，尽管非靶向化疗联合方案可改善反应，但对完全缓解和生存并无显著影响。齐多夫定与干扰素α联合的抗逆转录病毒疗法可诱导高比例的完全缓解并延长生存期，应作为急性成人T细胞白血病的首选治疗方案。成人T细胞淋巴瘤患者可能从初始的积极化疗后再进行抗逆转录病毒治疗中获益。为防止所有患者复发，可行时应强制进行异基因骨髓移植或额外的靶向治疗。基于当前的病理生理学，我们讨论了有前景的新药，如三氧化二砷、蛋白酶体抑制剂、维甲酸和血管生成抑制剂，以及细胞免疫疗法。
