Gilliam A C, Wood G S
Case Western Reserve University, University Hospitals of Cleveland, and Veterans Affairs Medical Center, OH 44106, USA.
Semin Oncol. 1999 Jun;26(3):290-306.
Primary cutaneous lymphomas present in and are confined to the skin with no evidence of extracutaneous disease. The skin is the second most common extranodal site involved by primary lymphoma; 50% are mycosis fungoides (MF)-type cutaneous T-cell lymphoma, with the remainder being peripheral T-cell lymphoma (25%) and B-cell lymphoma (25%). The diagnosis of non-MF primary cutaneous lymphomas differs from that of nodal lymphomas: (1) presentation in the skin more often predicts outcome than histology, (2) immunophenotyping and immunogenotyping studies show differences in chromosomal translocations, cell-surface antigen expression (T-cell receptor [TCR] and immunoglobulin [Ig] heavy and light chains), and oncogene expression, (3) involvement of structural compartments of the skin (epidermis, periadnexal or adventitial dermis, interstitial dermis, and subcutis) aids differential diagnosis in place of nodal architecture, and (4) cytokine and extracellular matrix environments may influence behavior of cutaneous lymphomas. Diagnosis often requires coordinated evaluation of clinical history, immunohistochemistry on paraffin and frozen sections of skin biopsies, and molecular analysis. Classification of primary cutaneous lymphomas by a combined histologic type and clinical behavior is useful.
原发性皮肤淋巴瘤起源于皮肤且局限于皮肤，无皮肤外疾病的证据。皮肤是原发性淋巴瘤累及的第二常见结外部位；50%为蕈样肉芽肿（MF）型皮肤T细胞淋巴瘤，其余为外周T细胞淋巴瘤（25%）和B细胞淋巴瘤（25%）。非MF原发性皮肤淋巴瘤的诊断与淋巴结淋巴瘤不同：（1）皮肤表现往往比组织学更能预测预后，（2）免疫表型和免疫基因分型研究显示染色体易位、细胞表面抗原表达（T细胞受体[TCR]和免疫球蛋白[Ig]重链和轻链）以及癌基因表达存在差异，（3）皮肤结构区室（表皮、毛囊周围或外膜真皮、间质真皮和皮下组织）的受累有助于替代淋巴结结构进行鉴别诊断，（4）细胞因子和细胞外基质环境可能影响皮肤淋巴瘤的行为。诊断通常需要综合评估临床病史、皮肤活检石蜡切片和冰冻切片的免疫组织化学以及分子分析。根据组织学类型和临床行为对原发性皮肤淋巴瘤进行分类是有用的。
