T-cell prolymphocytic leukemia with helper-cell phenotype and a review of the literature

The majority of published cases of prolymphocytic leukemia (PLL) have been of B-cell origin. Nineteen cases of PLL of T-cell type have been described, as has a single case of PLL having a surface phenotype with features of both B-cells and T-cells. This report presents a review of these cases and comparison with one case of T-cell PLL. By using specific monoclonal antibody technique, this case was subcategorized into helper-cell phenotype: E-rosette(+), SIG(-), Anti-T(+), Anti-B(-), Anti-monocyte(-), OKT3(+), OKT4(+), OKT6(-), OKT8(-), Ia(+), and Tdt(-). Cytochemical studies showed paranuclear acid phosphatase granules. Postmortem examination revealed a predominant T-cell zone infiltration by the leukemic cells in the spleen and lymph nodes, with involvement of multiple organs. The application of the monoclonal antibody technique, which can be standardized among different laboratories to subclassify lymphoproliferative disorders into functional subtypes, should lead to a better understanding and more effective treatment of this disease.