Marçais Ambroise, Suarez Felipe, Sibon David, Bazarbachi Ali, Hermine Olivier
Department of Hematology, Necker Hospital, 75473 Paris Cedex 15, France.
Leuk Res Treatment. 2012;2012:932175. doi: 10.1155/2012/932175. Epub 2012 Feb 14.
Adult T-cell leukaemia/lymphoma (ATLL) is an aggressive malignancy of mature activated T cells caused by human T-cell lymphotropic virus type I (HTLV-1). Prognosis is severe because of intrinsic chemoresistance and severe immuosuppression. Four different subtypes are described with different outcomes, and treatment strategies vary according to the different clinical courses. Japanese trials show that combinations of chemotherapy can increase the response rates especially in the lymphoma subtype. However, patients have a high rate of relapse and the outcome remains extremely poor. Recently, a worldwide meta-analysis demonstrated that the combination of Zidovudine and Interferon-alpha (IFN) is effective in the leukemic subtypes (smoldering, chronic, and acute) and influences favorably the course of the disease. In order to prevent relapse, clinical trials testing new drugs such as monoclonal antibodies or combinations such as arsenic/IFN are needed. Finally, allogeneic stem cell transplantation is a feasible option but bears a very high rate of complications.
成人T细胞白血病/淋巴瘤(ATLL)是一种由I型人类嗜T细胞病毒(HTLV-1)引起的成熟活化T细胞侵袭性恶性肿瘤。由于其内在的化疗耐药性和严重的免疫抑制,预后很差。根据不同的预后情况描述了四种不同的亚型,治疗策略也因不同的临床病程而异。日本的试验表明,化疗联合使用可提高缓解率,尤其是在淋巴瘤亚型中。然而,患者的复发率很高,预后仍然极差。最近,一项全球荟萃分析表明,齐多夫定和α干扰素(IFN)联合使用对白血病亚型(冒烟型、慢性型和急性型)有效,并对疾病进程有积极影响。为了预防复发,需要进行临床试验来测试新药,如单克隆抗体,或联合用药,如砷/IFN。最后,异基因干细胞移植是一种可行的选择,但并发症发生率非常高。