Gottlieb S L, Wolfe J T, Fox F E, DeNardo B J, Macey W H, Bromley P G, Lessin S R, Rook A H
Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia 19104, USA.
J Am Acad Dermatol. 1996 Dec;35(6):946-57. doi: 10.1016/s0190-9622(96)90119-x.
Extracorporeal photopheresis is a pheresis-based therapy that permits the direct targeting of psoralen-mediated photochemotherapy to circulating pathogenic T cells. Although photopheresis is currently used to treat cutaneous T-cell lymphoma (CTCL), limited data are available regarding overall response rates and durability of responses among patients with advanced disease. Furthermore, little is known about the effectiveness and tolerability of combined regimens employing other biologic response modifiers including interferon alfa.
Our purpose was to determine the efficacy of photopheresis among 41 patients with the clinical and laboratory diagnosis of CTCL; the majority of patients had stage III or IV disease with the presence of circulating malignant T cells.
A retrospective chart review during a 10-year period at a single university hospital was performed for all patients receiving either photopheresis monotherapy on two consecutive days every 4 weeks (one cycle) and for an additional 12 patients who also received interferon alfa 1.5 to 5 million U subcutaneously three to five times weekly.
Thirty-one of 41 patients (76%) were treated for six or more cycles. The remaining 10 were treated with less than six cycles because of rapidly progressing disease (n = 6), death unrelated to CTCL (n = 2), or withdrawal from treatment (n = 1); one of the 10 patients had only received five cycles of treatment but is still receiving therapy. Twenty-eight of the 31 patients treated for six or more cycles received photopheresis alone. Among the 28, seven patients (25%) had a complete remission, 13 (46%) had a partial remission defined as more than 50% clearing of skin disease, and eight (29%) did not respond to treatment. The presence of Sézary cells in the peripheral blood was associated with a favorable response. Median time to treatment failure was 18 months, whereas median survival from initiation of therapy was 77 months and from the time of diagnosis exceeded 100 months. Nine of these 28 patients went on to receive combination therapy with interferon alfa and, in some cases, other agents. Among these nine patients, five had an enhanced clinical response to the combination therapy compared with treatment with photopheresis monotherapy. The combined regimen was well tolerated.
These results indicate that patients with advanced CTCL can achieve a high response rate for an extended period with photopheresis and that interferon alfa combined with photopheresis is a well-tolerated regimen that appears to produce higher response rates than photopheresis alone.
体外光化学疗法是一种基于血液成分分离的治疗方法，可使补骨脂素介导的光化学疗法直接作用于循环中的致病性T细胞。尽管目前体外光化学疗法用于治疗皮肤T细胞淋巴瘤（CTCL），但关于晚期疾病患者的总体缓解率和缓解持久性的可用数据有限。此外，对于采用包括干扰素α在内的其他生物反应调节剂的联合治疗方案的有效性和耐受性知之甚少。
我们的目的是确定41例临床和实验室诊断为CTCL患者的体外光化学疗法的疗效；大多数患者为III期或IV期疾病，伴有循环恶性T细胞。
对一所大学医院10年间接受治疗的所有患者进行回顾性病历审查，这些患者要么每4周连续两天接受体外光化学疗法单一治疗（一个周期），另外12例患者还每周皮下注射150万至500万单位干扰素α三至五次。
41例患者中有31例（76%）接受了六个或更多周期的治疗。其余10例因疾病进展迅速（n = 6）、与CTCL无关的死亡（n = 2）或退出治疗（n = 1）而接受少于六个周期的治疗；10例患者中有1例仅接受了五个周期的治疗，但仍在接受治疗。接受六个或更多周期治疗的31例患者中有28例仅接受了体外光化学疗法。在这28例患者中，7例（25%）完全缓解，13例（46%）部分缓解，定义为皮肤疾病清除率超过50%，8例（29%）对治疗无反应。外周血中出现Sezary细胞与良好反应相关。治疗失败的中位时间为18个月，而从开始治疗的中位生存期为77个月，从诊断时起超过100个月。这28例患者中有9例继续接受干扰素α联合治疗，在某些情况下还联合其他药物。在这9例患者中，与单纯体外光化学疗法治疗相比，5例对联合治疗的临床反应增强。联合治疗方案耐受性良好。
这些结果表明，晚期CTCL患者通过体外光化学疗法可在较长时间内实现高缓解率，且干扰素α联合体外光化学疗法是一种耐受性良好的方案，似乎比单纯体外光化学疗法产生更高的缓解率。
