Nagatani T, Okazawa H, Mizuno H, Yanagi N, Miyazawa M, Baba N
Department of Dermatology, Yokohama City University School of Medicine, Japan.
Gan To Kagaku Ryoho. 1997 Jan;24(1):23-9.
Malignant lymphoma of the skin is a type of extranodal lymphoma with a benign prognosis, in which the main organ involved is the skin. Some 80-90% of the cases in Japan show a T-cell phenotype. Mycosis fungoides and Sézary syndrome are common T-cell lymphomas of the skin. The tumor cells of mycosis fungoides, small and medium-sized cells with cerebriform nuclei, are detected in an epidermo-dermo junction. The tumor cells show CD3, CD4 and CLA, (cutaneous lymphocyte associated antigen) positivity. Various forms of topical therapy, such as topical steroid, photochemotherapy (PUVA), and interferons, have been indicated for the good-risk group (stages I A, I B and II A). Electron-beam irradiation, various chemotherapy, such as low-dose etoposide, low-dose MTX and CPT-11 and deoxy coformycin (DCF) plus IFNs, have been indicated for intermediate-risk group (stage II B, III and IV A). BRMs plus low-dose etoposide, electron-beam irradiation and a multiagent combination chemotherapy, such as MACOP-B, M-BACOD or ProMACE-CytaBOM, have been indicated for the high-risk group (stages IV A and IV B). Cutaneous B cell lymphoma (CBCL) can be diagnosed using a molecular biological assay. The tumor cells of CBCL do not express T-cell antigens such as CD2, CD3 and CD43 and show B-cell antigens such as sIg, CD19, CD20 and CD22. Electron-beam irradiation has been indicated for early-stage CBCL (stages I and II). An effective multiagent combination chemotherapy, such as MACOP-B, M-BACOD or ProMACE-CytaBOM, is required for patients with advanced stage CBCL (stages III and IV).
皮肤恶性淋巴瘤是一种预后良好的结外淋巴瘤，主要累及器官为皮肤。在日本，约80%-90%的病例表现为T细胞表型。蕈样肉芽肿和赛塞里综合征是常见的皮肤T细胞淋巴瘤。蕈样肉芽肿的肿瘤细胞为中等大小、核呈脑回状的细胞，在表皮-真皮交界处被检测到。肿瘤细胞显示CD3、CD4和CLA（皮肤淋巴细胞相关抗原）阳性。对于低危组（ⅠA、ⅠB和ⅡA期），已采用多种局部治疗方法，如局部使用类固醇、光化学疗法（PUVA）和干扰素。对于中危组（ⅡB、Ⅲ和ⅣA期），已采用电子束照射、多种化疗方法，如低剂量依托泊苷、低剂量甲氨蝶呤和CPT-11以及脱氧助间型霉素（DCF）加干扰素。对于高危组（ⅣA和ⅣB期），已采用生物反应调节剂加低剂量依托泊苷、电子束照射和多药联合化疗，如MACOP-B、M-BACOD或ProMACE-CytaBOM。皮肤B细胞淋巴瘤（CBCL）可通过分子生物学检测进行诊断。CBCL的肿瘤细胞不表达T细胞抗原如CD2、CD3和CD43，而显示B细胞抗原如sIg、CD19、CD20和CD22。对于早期CBCL（Ⅰ和Ⅱ期），已采用电子束照射。对于晚期CBCL（Ⅲ和Ⅳ期）患者，需要有效的多药联合化疗，如MACOP-B、M-BACOD或ProMACE-CytaBOM。
