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Deoxycoformycin in the treatment of mature T-cell leukaemias.

机译:脱氧福尔霉素治疗成熟的T细胞白血病。

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摘要

We describe the results of treatment with 2'-deoxycoformycin (DCF) in 68 patients with post-thymic (mature) T-cell malignancies. These included: prolymphocytic leukaemia (T-PLL), 31, HTLV-1 + adult T-cell leukaemia/lymphoma (ATLL), 20, cutaneous T-cell lymphoma (CTCL), comprising mycosis fungoides and Sezary syndrome, 13, and large granular lymphocytic leukaemia, four. Two-thirds of patients were refractory to previous therapy, which included four drug combinations. DCF was given intravenously at 4 mg m-2 weekly for the first 4 weeks and then every 2 weeks until maximal response. Toxicity was very low with only one death resulting from prolonged neutropenia. Overall response rates, partial (PR) and complete. (CR), were 38%, with variations according to diagnosis. Best responses, 54%, were seen in CTCL but limited to Sezary patients, one CR, six PR, whilst none of the mycosis fungoides responded. Responses in T-PLL were recorded in 48% including three CR (of 8-12 months' duration unmaintained) and 12 PR. Fifteen per cent of responses were seen in ATLL. The only ATLL responders - two CR, one PR - were those patients who received combination chemotherapy prior to DCF, with reduction of tumour bulk but short of PR. When results were analysed according to membrane phenotypes it was apparent that responses were seen mainly in cases with CD4+, CD8- T cells -22 of 47 (47%) - contrasting with only three of 19 (16%) with other T-cell phenotypes. We conclude that DCF is a useful therapy for the treatment of T-cell leukaemias, in particular Sezary syndrome and T-PLL, and should play a part in strategies to improve the natural history of this group of lymphoid malignancies.
机译:我们描述了在胸腺后(成熟)T细胞恶性肿瘤68例患者中,用2'-脱氧考福霉素(DCF)治疗的结果。其中包括:前淋巴细胞性白血病(T-PLL),31,HTLV-1 +成人T细胞白血病/淋巴瘤(ATLL),20,皮肤性T细胞淋巴瘤(CTCL),包括蕈样肉芽肿和Sezary综合征,13,大粒状淋巴细胞白血病四。三分之二的患者对以前的治疗无效,其中包括四种药物组合。在最初的4周中,每周4 mg m-2静脉内给予DCF,然后每2周静脉给予DCF,直至达到最大反应。毒性非常低,仅因长期中性粒细胞减少症而死亡。总体响应率,部分(PR)和完整。 (CR)为38%,根据诊断有所不同。在CTCL中观察到最佳反应,为54%,但仅限于Sezary患者,1例CR,6例PR,而真菌病菌均无反应。 T-PLL的应答记录为48%,包括三个CR(维持8-12个月的持续时间)和12个PR。 ATLL中有15%的答复。仅有ATLL反应者-2例CR,1例PR-是那些在DCF之前接受联合化疗,肿瘤体积减少但PR不足的患者。根据膜表型分析结果时,很明显,主要在CD4 +,CD8- T细胞-22为47(47%)的情况下观察到反应-与其他T细胞表型中只有19个中的三个(16%)形成对比。我们得出结论,DCF是治疗T细胞白血病(尤其是Sezary综合征和T-PLL)的有用疗法,并应在改善这一类淋巴恶性肿瘤自然史的策略中发挥作用。

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