[Primary cutaneous lymphoma--mycosis fungoides].

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).

[Lymphomas of the skin].

Malignant lymphoma of the skin is a type of extranodal lymphoma, in which the main organ involved is the skin, and 80-90% of cases in Japan show a T-cell phenotype. Mycosis fungoides (MF) and Sézary syndrome (SS) are common T-cell lymphomas of the skin with a benign prognosis. Therefore, various forms of topical therapy, such as topical steroid, photochemotherapy (PUVA) and interferons, have been indicated for the low-risk group (stages I A, I B and II A), whereas electron-beam irradiation, retinoid plus interferon (IFN), photopheresis and deoxycoformycin (DCF) plus IFN have been indicated for intermediate-risk group (stages II B and III). The cutaneous involvement of B-cell lymphoma has been considered an unmistakable sign of progression and dissemination of lymphoid disease, and is thus associated with a poor prognosis. However, some primary cutaneous B-cell lymphomas (CBCLs) show a benign prognosis, and electron-beam irradiation has been indicated for early-stage CBCL (stages I and II). However, the prognosis of high-risk group CTCL (stage IV) and cutaneous B-cell lymphoma (CBCL) (stages III and IV) is poor. Therefore, an effective multiagent combination chemotherapy, such as MACOP-B, M-BACOD or ProMACE-Cyta BOM is required for patients with advanced-stage CTCL and CBCL. With regard to age at the time of the first medical examination, patients with CTCL or CBCL at an advanced stage have a tendency to be older. Therefore, a mild but effective therapy, such as DCF plus IFN is recommendable.