T-cell prolymphocytic leukemia with an unusual phenotype CD4+ CD8+.
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.
Kluin-Nelemans
H C
HC
Gmelig-Meyling
F H
FH
Kootte
A M
AM
den Ottolander
G J
GJ
Termijtelen
A
A
Kluin
P M
PM
Beverstock
G C
GC
Brand
A
A
D000368:Aged; D000911:Antibodies, Monoclonal; D005260:Female; D005455:Fluorescent Antibody Technique; D006651:Histocytochemistry; D006801:Humans; D007376:Interleukin-2 / Q000096:biosynthesis; D007945:Leukemia, Lymphoid / Q000235:genetics* / Q000628:therapy; D007958:Leukocyte Count; D008213:Lymphocyte Activation; D010641:Phenotype; D013154:Spleen / Q000276:immunology; D013601:T-Lymphocytes / Q000145:classification* / Q000276:immunology / Q000648:ultrastructure
D000911:Antibodies, Monoclonal; D007376:Interleukin-2