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儿童蕈样肉芽肿:28例患者的经验及对光疗的反应

Childhood mycosis fungoides: experience of 28 patients and response to phototherapy.

作者信息

Laws Philip M, Shear Neil H, Pope Elena

机构信息

Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

出版信息

Pediatr Dermatol. 2014 Jul-Aug;31(4):459-64. doi: 10.1111/pde.12338. Epub 2014 Jun 11.

Abstract

Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma (CTCL), is rare in childhood. The prognosis and response to treatment are poorly described in children. The objective of the current study was to evaluate the response to phototherapy in a pediatric cohort. A retrospective cohort study of all patients diagnosed with MF before the age of 18 years and referred to the regional CTCL phototherapy service was performed between January 1990 and April 2012. Twenty-eight patients were identified (13 boys, 15 girls). The mean age at presentation was 11.6 ± 3.9 years. The hypopigmented variant was noted in 79% of patients. All patients had stage I disease (IA = 10, IB = 17, unknown = 1). The median follow-up after diagnosis was 43 months (range 6-274 mos). Narrowband ultraviolet B (NbUVB; 311 nm) was used as first-line phototherapy in 18 patients and psoralen (bath) plus ultraviolet A (PUVA) was used in 8 patients. Complete or partial response was observed in 19 of 22 patients (86%). A further course of phototherapy was required in 7 of 12 patients (58%) treated with NbUVB after a median of 4 months (range 4-29 mos). A further course of phototherapy was required in four of eight patients (50%) successfully treated with PUVA after a median of 45.5 months (range 30-87 mos). No disease progression was noted over the follow-up (median 43 mos). The majority of patients in our cohort had hypopigmented MF. Phototherapy offers an effective option for treatment of childhood MF, although the period of remission may be greater in patients treated with PUVA.

摘要

蕈样肉芽肿(MF)是最常见的皮肤T细胞淋巴瘤(CTCL),在儿童中较为罕见。儿童患者的预后及对治疗的反应鲜有描述。本研究的目的是评估一组儿科患者对光疗的反应。对1990年1月至2012年4月期间所有18岁前诊断为MF并转诊至地区CTCL光疗服务中心的患者进行了一项回顾性队列研究。共确定了28例患者(13名男孩,15名女孩)。就诊时的平均年龄为11.6±3.9岁。79%的患者为色素减退型。所有患者均为I期疾病(IA = 10例,IB = 17例,分期不明 = 1例)。诊断后的中位随访时间为43个月(范围6 - 274个月)。18例患者将窄谱中波紫外线(NbUVB;311nm)作为一线光疗,8例患者使用补骨脂素(浴用)加紫外线A(PUVA)。22例患者中有19例(86%)观察到完全或部分缓解。接受NbUVB治疗的12例患者中有7例(58%)在中位4个月(范围4 - 29个月)后需要进一步的光疗疗程。接受PUVA成功治疗的8例患者中有4例(50%)在中位45.5个月(范围30 - 87个月)后需要进一步的光疗疗程。随访期间(中位43个月)未观察到疾病进展。我们队列中的大多数患者为色素减退型MF。光疗为儿童MF的治疗提供了一种有效的选择,尽管接受PUVA治疗的患者缓解期可能更长。

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