T-cell prolymphocytic leukemia with an unusual phenotype CD4+ CD8

• Published in: Cancer Vol. 60; no. 4; p. 794
• Main Authors: Kluin-Nelemans, H C, Gmelig-Meyling, F H, Kootte, A M, den Ottolander, G J, Termijtelen, A, Kluin, P M, Beverstock, G C, Brand, A
• Format: Journal Article
• Language: English
• Published: United States 15-08-1987
• Subjects: Aged; Antibodies, Monoclonal; Female; Fluorescent Antibody Technique; Histocytochemistry; Humans; Interleukin-2 - biosynthesis; Leukemia, Lymphoid - genetics; Leukemia, Lymphoid - therapy; Leukocyte Count; Lymphocyte Activation; Phenotype; Spleen - immunology; T-Lymphocytes - classification; T-Lymphocytes - immunology; T-Lymphocytes - ultrastructure
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(19870815)60:4<794::AID-CNCR2820600415>3.0.CO;2-R

A patient with T-cell prolymphocytic leukemia (T-PLL) is described. The outcome was poor, with death 8 months after diagnosis, despite several therapeutic interventions. The cells carried both CD4 and CD8 epitopes, but other thymocytic markers were absent. The spleen showed infiltration of CD4+ CD8+ prolymphocytes in the red pulp and in T-cell-dependent areas of the white pulp. Immunologic function studies revealed proliferation after stimulation with mitogens and even several antigens. However, in the mixed lymphocyte culture the T-PLL cells did not proliferate. Cytotoxic T-cells could not be induced. In T-non-T recombination experiments neither helper nor suppressor cell function was found for pokeweed mitogen-dependent plasmablast generation of normal B-cells. Cytogenetically, many abnormalities were found. Among them, 14q+; absence of chromosomes 8, 11, and 22; and the presence of large marker chromosomes and fragments.