K. Heinonen et al., MULTIPLE KARYOTYPIC ABNORMALITIES IN 3 CASES OF SMALL-CELL VARIANT OFT-CELL PROLYMPHOCYTIC LEUKEMIA, Cancer genetics and cytogenetics, 78(1), 1994, pp. 28-35
Cytogenetic, clinical, and laboratory findings of three patients with
a small cell variant of T-cell prolymphocytic leukemia (T-PLL) are pre
sented. Immunophenotypic studies of the morphologically typical small
cell variant prolymphocytes showed a mature helper T-cell phenotype (C
D4(+) CD8(-)) in one patient and a common thymocyte phenotype (CD4(+)
CD8(+)) in two other patients. The cytogenetic analysis revealed compl
ex karyotypes with several structural aberrations in the peripheral bl
ood lymphocytes of all three patients. In all cases chromosome 14 was
affected with the breakpoint at 14q11. Inversion (14) and isochromosom
e 8q, often reported as an additional aberration in T-PLL, were detect
ed in two of the patients. In two patients a translocation of the shor
t arm of chromosome 12 was also seen. The T-ceII receptor beta-chain g
ene showed a clonal rearrangement in all three patients, whereas no re
arrangements were detected in the immunoglobulin genes. The survival o
f the patients ranged from 10 weeks to 48 months. The association betw
een cytogenetic, clinical, and laboratory data is discussed.