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Clinical Trials and Treatment of ATL

机译：ATL的临床试验和治疗

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ATL is a distinct peripheral T-lymphocytic malignancy associated with human T-cell lymphotropic virus type I (HTLV-1). The diversity in clinical features and prognosis of patients with this disease has led to its subtype-classification into four categories, acute, lymphoma, chronic, and smoldering types, defined by organ involvement, and LDH and calcium values. In case of acute, lymphoma, or unfavorable chronic subtypes (aggressive ATL), intensive chemotherapy like the LSG15 regimen (VCAP-AMP-VECP) is usually recommended if outside of clinical trials, based on the results of a phase 3 trial. In case of favorable chronic or smoldering ATL (indolent ATL), watchful waiting until disease progression has been recommended, although the long-term prognosis was inferior to those of, for instance, chronic lymphoid leukemia. Retrospective analysis suggested that the combination of interferon alpha and zidovudine was apparently promising for the treatment of ATL, especially for types with leukemic manifestation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is also promising for the treatment of aggressive ATL possibly reflecting graft versus ATL effect. Several new agent trials for ATL are ongoing and in preparation, including a defucosylated humanized anti-CC chemokine receptor 4 monoclonal antibody, IL2-fused with diphtheria toxin, histone deacetylase inhibitors, a purine nucleoside phosphorylase inhibitor, a proteasome inhibitor, and lenalidomide.
机译：ATL是与人类I型淋巴细胞性病毒（HTLV-1）相关的独特的外周性T淋巴细胞恶性肿瘤。该疾病患者的临床特征和预后的多样性已导致其亚型分为四类，急性，淋巴瘤，慢性和阴燃类型，由器官受累，LDH和钙值定义。如果是急性，淋巴瘤或不利的慢性亚型（攻击性ATL），则根据3期试验的结果，如果不在临床试验范围内，通常建议进行强力化疗，例如LSG15方案（VCAP-AMP-VECP）。对于长期或阴燃的ATL（惰性ATL），建议长期观察直至疾病进展，尽管长期预后不如慢性淋巴白血病。回顾性分析表明，干扰素α和齐多夫定的联合治疗有望治疗ATL，特别是对于具有白血病表现的类型。同种异体造血干细胞移植（allo-HSCT）也有望用于治疗侵袭性ATL，可能反映了移植物抗ATL的作用。 ATL的一些新药物试验正在进行中，包括在准备中，包括去岩藻糖基化的人源化抗CC趋化因子受体4单克隆抗体，与白喉毒素融合的IL2，组蛋白脱乙酰基酶抑制剂，嘌呤核苷磷酸化酶抑制剂，蛋白酶体抑制剂和来那度胺。

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