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

Abstract

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.

T‐cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: A distinct clinicopathologic subtype with a poor prognosis

“…Prolymphocytic leukemia is usually a B-cell malignant disorder, but patients with T-cell prolymphocytic leukemia (T-PLL) have also been reported [2,17,19,20,22,24,[27][28][29]331. The majority of these reported patients with T-PLL who were studied with monoclonal antibodies had a T4 + /T8 -cell membrane phenotype.…”

Functional Properties of Neoplastic T Cells in Helper T Cell Prolymphocytic Leukaemia with IgM Hypergammaglobulinaemia

“…They have varied in clinical, morphological, and functional characteristics. Tsai et al [19], Knowles [8], and Foon & Todd [5], have presented a good review of these cases.…”

“…Since the description by Galton et al [3,6], several reports of prolymphocytic leukaemia have been published [4,7,17,18], Whereas prolymphocytic leukaemia can be distinguished from typical chronic lymphocytic leukaemia (CLL) by its clinicopathological features [5,8], it is generally considered to be a variant of CLL, The clinical, haematological and ultrastructural features of the B and T cell prolymphocytic leukaemia cases have been described [4,18], There do not appear to be major clinical or haematological differences between the B and T cell prolymphocytic leukemia [5,19], In T cell prolymphocytic leukaemia, most cells express the mature helper/inducer (T3+ T4+ T8") or the mature suppressor/cytotoxic (T3* T4" T8*) phenotype, but occasionally the cells are T4+ T8+ or T4-T8- [5,8], A few cases of T cell prolymphocytic leukaemia of the T4+ helper/ inducer T cell phenotype have been studied as regards ultrastructure and cytochemistry, but the details of multiparametric studies using specific monoclonal antibodies and functional studies have not been reported. In this report, we describe phenotypic marker and imtnunologic functional studies of a prolymphocytic leukaemia patient.…”