Lauria F, Foà R, Raspadori D, Tazzari P L, Migone N, Giubellino M C, Lusso P, Fierro M T, Motta M R, Tassinari A
Istituto di Ematologia, L. e A. Seràgnoli, University of Bologna, Italy.
Eur J Haematol. 1987 Jul;39(1):1-6. doi: 10.1111/j.1600-0609.1987.tb00154.x.
In this study we describe 2 patients who appear to suffer from a morphological, cytochemical and clinico-haematological variant of T-prolymphocytic leukaemia (T-PLL). The cells were smaller than typical prolymphocytes, with a regular nucleus containing a smaller and less prominent nucleolus; the alpha-naphthyl acetate esterase (ANAE) and acid phosphatase (AP) cytochemical reactions showed a weaker pattern of positivity in this variant compared to T-PLL. No immunological differences were found between the two conditions with regard to membrane expression and functional behavior of the cells. The clinical course and the outcome of the patients appears to be different: aggressive and rapidly fatal in T-PLL; thus far well-controlled in the T-PLL variant. From a molecular point of view, both cases showed a monoclonal rearrangement of the T-cell receptor beta-chain gene.
在本研究中,我们描述了2例似乎患有T-原淋巴细胞白血病(T-PLL)的形态学、细胞化学和临床血液学变异型患者。这些细胞比典型的原淋巴细胞小,细胞核规则,核仁较小且不明显;与T-PLL相比,α-萘乙酸酯酶(ANAE)和酸性磷酸酶(AP)细胞化学反应在该变异型中显示出较弱的阳性模式。在细胞的膜表达和功能行为方面,两种情况之间未发现免疫学差异。患者的临床病程和结局似乎不同:T-PLL呈侵袭性且迅速致命;到目前为止,T-PLL变异型病情得到良好控制。从分子角度来看,两例均显示T细胞受体β链基因的单克隆重排。