Hymes Kenneth B
New York University, Cancer Institute, New York 10016, USA.
Oncology (Williston Park). 2007 Feb;21(2 Suppl 1):18-23.
Mycosis fungoides is responsive to treatment in the early stages; patients have a long duration of survival but are rarely cured of the disease. Therefore, patients require long-term, sequential therapies with as little toxicity as possible. In the early stages, skin-directed therapies, such as psoralen plus ultraviolet A in combination with retinoids or interferon, generally produce good, long-term responses. Once the disease progresses, systemic agents such as cytokines and retinoids are introduced. The cytokines provide a rational treatment approach for cutaneous T-cell lymphoma (CTCL) and produce good, long-lasting responses with few immunosuppressant effects. Denileukin diftitox (Ontak) has also been shown to produce good treatment effects, and its toxic effects can usually be controlled using prophylactic therapies. The synthetic retinoid bexarotene (Targretin) is taken orally and produces high response rates in CTCL, with a good long-term tolerability profile. Conventional systemic chemotherapies produce rapid responses and high response rates in CTCL, but these are generally of short duration and accompanied by myelosuppression and immunosuppression. Current treatment strategies therefore consist of the use of initial skin-directed therapies, with the addition of low-toxicity systemic biologic agents as the disease progresses; patients who do not respond to biologic agents should then receive conventional chemotherapies, starting with single agents and progressing to combination therapies.
蕈样肉芽肿在疾病早期对治疗有反应;患者生存期较长,但很少能治愈。因此,患者需要长期、序贯的治疗,且毒性尽可能小。在疾病早期,针对皮肤的治疗方法,如补骨脂素加紫外线A联合维甲酸或干扰素,通常能产生良好的长期疗效。一旦疾病进展,就会引入细胞因子和维甲酸等全身用药。细胞因子为皮肤T细胞淋巴瘤(CTCL)提供了一种合理的治疗方法,能产生良好且持久的疗效,免疫抑制作用较小。地尼白介素-妥昔单抗(Ontak)也已显示出良好的治疗效果,其毒性作用通常可通过预防性治疗加以控制。合成维甲酸贝沙罗汀(Targretin)口服给药,在CTCL中产生的缓解率较高,长期耐受性良好。传统的全身化疗在CTCL中能产生快速反应和高缓解率,但这些反应通常持续时间较短,并伴有骨髓抑制和免疫抑制。因此,目前的治疗策略包括首先采用针对皮肤的治疗方法,随着疾病进展添加低毒性的全身生物制剂;对生物制剂无反应的患者应接受传统化疗,从单药开始,逐步过渡到联合治疗。