Hödl S
Z Hautkr. 1986 Jul 15;61(14):993-1013.
47 patients suffering from mycosis fungoides and 13 with Sézary's syndrome were retrospectively studied with regard to prognosis in relation to the histologic substrate of cutaneous lesions. On account of the wide spectrum of T-cell cytomorphology as well as the pattern and composition of the infiltrate, we distinguished 4 histopathological types: small cell lymphoid type (45%), mixed cell polymorphic type (40%), large cell type (immunoblastic and pleomorphic, 10%), and medium-sized cell type (5%). Statistical analysis revealed survival rates of 10 years in 68.3% for the small cell lymphoid type and in 30.3% for the mixed cell polymorphic type. All patients showing the large cell type died within 6 years after diagnosis; those bearing the medium-sized cell type died within 2 years after diagnosis. The forms of mycosis fungoides showing the last two types of infiltrate may be classified as lymphomas of extreme malignancy. The different histological features are not only significant with regard to the diagnosis but also permit prognostic estimation before systemic signs have become evident.
对47例蕈样肉芽肿患者和13例塞扎里综合征患者进行了回顾性研究,以探讨皮肤病变组织学基础与预后的关系。鉴于T细胞细胞形态的广泛谱系以及浸润的模式和组成,我们区分出4种组织病理学类型:小细胞淋巴样型(45%)、混合细胞多形型(40%)、大细胞型(免疫母细胞型和多形型,10%)和中等大小细胞型(5%)。统计分析显示,小细胞淋巴样型10年生存率为68.3%,混合细胞多形型为30.3%。所有表现为大细胞型的患者在诊断后6年内死亡;表现为中等大小细胞型的患者在诊断后2年内死亡。表现出后两种浸润类型的蕈样肉芽肿形式可归类为极恶性淋巴瘤。不同的组织学特征不仅对诊断具有重要意义,而且在全身症状明显之前就可以进行预后评估。