V. Britobabapulle et al., RELATIONSHIP OF T-LEUKEMIAS WITH CEREBRIFORM NUCLEI TO T-PROLYMPHOCYTIC LEUKEMIA - A CYTOGENETIC ANALYSIS WITH IN-SITU HYBRIDIZATION, British Journal of Haematology, 96(4), 1997, pp. 724-732
Sezary cell leukaemia (SCL) is a mature T-cell leukaemia with characte
ristic cerebriform nuclei, whereas Sezary syndrome (SS) involves a mat
ure T-cell lymphoma with a similar nuclear morphology, We have examine
d these diseases by cytogenetics, chromosome painting and fluorescence
in situ hybridization (FISH). Both diseases had complex cytogenetic a
bnormalities. AU three cases of SCL investigated had inv(14)(q11;q32)
and two had iso(8q). No case of SS had these abnormalities but, instea
d, 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 rath
er than of SS.