儿童皮肤淋巴瘤的治疗进展

Treatment of pediatric cutaneous lymphomas

儿童皮肤淋巴瘤是指原发于皮肤的有明显异质性的一组淋巴增殖性疾病，分类主要依据WHO-EORTC和WHO系统。最常见类型为皮肤成熟T细胞淋巴瘤与皮肤成熟B细胞淋巴瘤。儿童皮肤淋巴瘤罕见，因临床表现与成人有差异及临床试验少、用药更慎重等原因，治疗方法主要基于个案报道。治疗包括局部外用药、光疗、放化疗、手术、干细胞移植及新型药物治疗等。各类型皮肤淋巴瘤的治疗方法与疗效差异大，保守治疗在大多数儿童期蕈样肉芽肿以及其他惰性生物学特征的皮肤淋巴瘤中有效，具有侵袭性生物学特征的皮肤淋巴瘤仍需化疗、靶向治疗等。侵袭性皮肤淋巴瘤在儿童较成人比例高．需雷视系统评估与随访观察．

Pediatric cutaneous lymphomas are a group of lymphoproliferative diseases with obvious heterogeneity, which are primarily derived from the skin. The classification is mainly based on the WHO- EORTC and WHO systems. The most common types of primary cutaneous lymphomas in children are cutaneous mature T-cell lymphomas （CTCL） and cutaneous mature B-cell lymphomas （CBCL）. Pediatric cutaneous lymphomas are rare, and their treatment approaches are mainly based on some case reports because of their clinical manifestations different from those of adults, few clinical trials and cautious medication. Treatments include topical drugs, phototherapy, radiotherapy, chemotherapy, surgery, stem cell transplantation and some new drugs. There are great differences in the therapeutic approaches and responses among various types of cutaneous lymphomas. Conservative treatment is effective in most childhood mycosis fungoides and other cutaneous lymphomas with inert biological features, while invasive cutaneous lymphomas still require chemotherapy and targeted therapies. Invasive cutaneous lymphomas occur more frequently in children than adults, so more attention should he paid to systematic assessment and follow-up observation,