Aggressive acute CD3+, CD56- T cell large granular lymphocyte leukemia with two stages of maturation arrest.
In the majority of clonal expansions of CD3+ large granular lymphocytes (LGL), referred to as T-LGL leukemia, patients have a chronic disease, often manifested by severe neutropenia, rheumatoid arthritis, and mild to moderate splenomegaly. The characteristic leukemic phenotype is CD3+, CD8+, CD16+, CD57+ and CD56-. Here we report an unusual case of T-LGL (CD3cyt+, CD3surface-, CD16+, CD56-) with clinicopathological features (acute presentation, large tumor mass, and systemic illness with highLGL counts at diagnosis) similar to those described for patients with CD3-natural killer (NK)-LGL leukemia. Two distinct stages of maturation arrest were observed: in the lymph node abnormal cells were CD4+, CD8+ whereas the majority of circulating leukemic cells expressed only CD8. TCR gamma (TCR gamma) gene configuration demonstrated that these originated from the same T cell clone, suggesting a maturation process between the two populations, or preferential passage of CD8 single positive cells into the blood.
Tordjman
R
R
Department of Clinical and Biological Hematology, Hopital Necker, Paris, France.
Macintyre
E
E
Emile
J F
JF
Valensi
F
F
Ribrag
V
V
Burtin
M L
ML
Varet
B
B
Brousse
N
N
Hermine
O
O
D000293:Adolescent; D001483:Base Sequence; D017252:CD3 Complex / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D002453:Cell Cycle / Q000502:physiology; D002999:Clone Cells; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D015458:Leukemia, T-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008113:Liver Neoplasms / Q000473:pathology; D008207:Lymphatic Metastasis; D008969:Molecular Sequence Data; D013160:Splenic Neoplasms / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology
D017252:CD3 Complex; D019002:CD56 Antigen