Brito-Babapulle V, Maljaie S H, Matutes E, Hedges M, Yuille M, Catovsky D
Royal Marsden Hospital, London.
Br J Haematol. 1997 Mar;96(4):724-32. doi: 10.1046/j.1365-2141.1997.9702605.x.
Sezary cell leukaemia (SCL) is a mature T-cell leukaemia with characteristic cerebriform nuclei, whereas Sezary syndrome (SS) involves a mature T-cell lymphoma with a similar nuclear morphology. We have examined these diseases by cytogenetics chromosome painting and fluorescence in situ hybridization (FISH). Both diseases had complex cytogenetic abnormalities. All three cases of SCL investigated had inv(14)(q11:q32) and two had iso(8q). No case of SS had these abnormalities but, instead, iso(17q) or 17p+ was present in the three cases of SS investigated and FISH indicated loss of heterozygosity due to deletion of a region at 17p 13 that included the tumour suppressor gene P53, implicating it in this malignancy. One case of SCL had iso(17q). The abnormalities of chromosomes 8 and 14 in SCL are commonly observed in T-prolymphocytic leukaemia (T-PLL) and suggest that SCL may be a variant of T-PLL rather than of SS.
Sezary细胞白血病(SCL)是一种具有特征性脑回状核的成熟T细胞白血病,而Sezary综合征(SS)则是一种具有相似核形态的成熟T细胞淋巴瘤。我们通过细胞遗传学染色体描绘和荧光原位杂交(FISH)对这些疾病进行了研究。两种疾病都有复杂的细胞遗传学异常。所研究的3例SCL均有inv(14)(q11:q32),2例有iso(8q)。所研究的3例SS均无这些异常,但取而代之的是,所研究的3例SS中有iso(17q)或17p+,FISH显示由于17p13区域缺失导致杂合性丢失,该区域包含肿瘤抑制基因P53,提示其与这种恶性肿瘤有关。1例SCL有iso(17q)。SCL中8号和14号染色体的异常在T-原淋巴细胞白血病(T-PLL)中常见,提示SCL可能是T-PLL的一个变异型,而非SS的变异型。