Matutes E, Brito-Babapulle V, Worner I, Sainati L, Foroni L, Catovsky D
MRC Leukaemia Unit, Royal Postgraduate Medical School, London, UK.
Nouv Rev Fr Hematol (1978). 1988;30(5-6):347-51.
Mature (post-thymic) T-cell malignancies comprise a heterogeneous group of diseases with distinct clinical, morphological and immunophenotypic features. As some of these features overlap in the various disorders, it is essential to use a number of diagnostic criteria to define the clinicopathological entities, namely: membrane markers, morphology by light and electron microscopy, clinical features, seroepidemiology of HTLV-I, histopathology, cytogenetic studies and DNA analysis. The latter, which is necessary to document clonality and confirm T-lineage, examines the rearrangement of the T-cell receptor beta, gamma and delta genes. By means of this multiparameter approach, it is possible to distinguish within the post-thymic leukaemias 4 disease entities: T-chronic lymphocytic leukaemia or large granular lymphocyte leukaemia, T-prolymphocytic leukaemia, adult T-cell leukaemia/lymphoma and Sézary syndrome.
成熟（胸腺后）T细胞恶性肿瘤是一组异质性疾病，具有不同的临床、形态学和免疫表型特征。由于这些特征在各种疾病中存在重叠，因此必须使用多种诊断标准来定义临床病理实体，即：膜标志物、光镜和电镜下的形态学、临床特征、HTLV-I血清流行病学、组织病理学、细胞遗传学研究和DNA分析。后者对于记录克隆性和确认T细胞谱系是必要的，它检查T细胞受体β、γ和δ基因的重排。通过这种多参数方法，可以在胸腺后白血病中区分出4种疾病实体：T慢性淋巴细胞白血病或大颗粒淋巴细胞白血病、T原淋巴细胞白血病、成人T细胞白血病/淋巴瘤和蕈样肉芽肿综合征。
