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采用补骨脂素加紫外线A光单药疗法实现完全缓解的早期皮肤T细胞淋巴瘤患者的长期随访

Long-term follow-up of patients with early-stage cutaneous T-cell lymphoma who achieved complete remission with psoralen plus UV-A monotherapy.

作者信息

Querfeld Christiane, Rosen Steven T, Kuzel Timothy M, Kirby Katharine A, Roenigk Henry H, Prinz Bettina M, Guitart Joan

机构信息

Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA.

出版信息

Arch Dermatol. 2005 Mar;141(3):305-11. doi: 10.1001/archderm.141.3.305.

Abstract

OBJECTIVES

To evaluate long-term outcomes, impact of maintenance therapy and potential curability of patients with mycosis fungoides (MF) treated with psoralen plus UV-A (PUVA) monotherapy.

DESIGN

Single-center retrospective cohort analysis.

SETTING

Academic referral center for cutaneous lymphoma.

PATIENTS

A total of 66 of 104 patients with clinical stages IA to IIA MF who achieved complete remission (CR) after PUVA monotherapy between 1979 and 1995.

MAIN OUTCOME MEASURES

Kaplan-Meier actuarial survival and disease-free survival curves were compared between stage IA and IB/IIA cases. Patients were stratified into relapse and nonrelapse groups based on whether their MF relapsed during study follow-up. Baseline characteristics and treatment were compared between these groups.

RESULTS

Median follow-up time was 94 months (range, 5-242 months). Thirty-three patients maintained CR for 84 months (range, 5-238 months), and 33 patients experienced relapse with a median disease-free interval of 39 months (range, 2-127 months). There was no significant difference in baseline characteristics between patients in the nonrelapse and relapse groups. Those in the nonrelapse group received a higher cumulative dosage to CR (P = .03) and required longer treatment periods to achieve CR (P = .03). Disease-free survival rates at 5 and 10 years for all patients with stage IA were 56% and 30%, respectively, and for stage IB/IIA, 74% and 50%. Actuarial survival rates at 5, 10, and 15 years were 94%, 82%, and 82%, respectively, in patients with stage IA, and 80%, 69%, and 58% in patients with stage IB/IIA. The overall survival rate for the nonrelapse and relapse groups did not show any statistical difference. One third of the patients developed signs of chronic photodamage and secondary cutaneous malignancies.

CONCLUSIONS

Psoralen UV-A is an effective treatment for MF, inducing long-term remissions and perhaps in some cases disease "cure." Thirty percent to 50% of patients remain disease free for 10 years, but late relapses occur. Long-term survival is not affected by relapse status, and the risk of photodamage needs to be measured against the possible benefit of greater disease elimination.

摘要

目的

评估补骨脂素加紫外线A(PUVA)单一疗法治疗蕈样肉芽肿(MF)患者的长期疗效、维持治疗的影响及潜在治愈率。

设计

单中心回顾性队列分析。

地点

皮肤淋巴瘤学术转诊中心。

患者

1979年至1995年间,104例临床IA至IIA期MF患者中,共有66例在接受PUVA单一疗法后达到完全缓解(CR)。

主要观察指标

比较IA期和IB/IIA期病例的Kaplan-Meier精算生存率和无病生存率曲线。根据患者在研究随访期间MF是否复发,将其分为复发组和未复发组。比较两组的基线特征和治疗情况。

结果

中位随访时间为94个月(范围5 - 242个月)。33例患者维持CR达84个月(范围5 - 238个月),33例患者复发,无病间隔中位时间为39个月(范围2 - 127个月)。未复发组和复发组患者的基线特征无显著差异。未复发组患者达到CR时接受的累积剂量更高(P = 0.03),达到CR所需的治疗时间更长(P = 0.03)。所有IA期患者5年和10年的无病生存率分别为56%和30%,IB/IIA期分别为74%和50%。IA期患者5年、10年和15年的精算生存率分别为94%、82%和82%,IB/IIA期患者分别为80%、69%和58%。未复发组和复发组的总生存率无统计学差异。三分之一的患者出现慢性光损伤和继发性皮肤恶性肿瘤迹象。

结论

补骨脂素紫外线A是治疗MF的有效方法,可诱导长期缓解,在某些情况下可能治愈疾病。30%至50%的患者10年无病,但会出现晚期复发。长期生存不受复发状态影响,需要权衡光损伤风险与更大程度消除疾病的可能益处。

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