Relationship of T leukaemias with cerebriform nuclei to T-prolymphocytic leukaemia: a cytogenetic analysis with in situ hybridization

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 17p13 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.