T-cell receptor tau delta +/CD3+4-8-T- cell acute lymphoblastic leukemias: a distinct subgroup of leukemias in children. A report of five cases.
In a 10-year study of T-cell acute lymphoblastic leukemias (T-ALL) in children, we have identified five cases expressing the T-cell receptor tau delta (TCR tau delta). The incidence (26%) of TCR tau delta+T-cell leukemias in our material was high. Clinically, the TCR tau delta+ leukemias represented a distinct subgroup of T-cell leukemias. Mean age at onset of disease, 1.8 years, was remarkably low for mature T-cell leukemias. White blood cell counts were high, lymph node enlargements were discrete, and no mediastinal tumors were seen. Four of five TCR tau delta+ leukemias carried rearrangements of the C tau 2 gene, and transcribed the T-early alpha genetic element.
Alfsen
G C
GC
Department of Pathology, Norwegian Radium Hospital, Oslo.
Beiske
K
K
Holte
H
H
Hovig
E
E
Deggerdal
A
A
Sandlie
I
I
Widing
E
E
Slørdahl
S
S
Klepper
L K
LK
Sizoo
W
W
D015703:Antigens, CD / Q000032:analysis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D017252:CD3 Complex; D015704:CD4 Antigens / Q000032:analysis; D016827:CD8 Antigens; D002648:Child; D002675:Child, Preschool; D015342:DNA Probes; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000145:classification / Q000276:immunology* / Q000473:pathology; D008297:Male; D009693:Nucleic Acid Hybridization; D016133:Polymerase Chain Reaction; D012333:RNA, Messenger / Q000378:metabolism; D011948:Receptors, Antigen, T-Cell / Q000032:analysis / Q000235:genetics / Q000378:metabolism*
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D017252:CD3 Complex; D015704:CD4 Antigens; D016827:CD8 Antigens; D015342:DNA Probes; D012333:RNA, Messenger; D011948:Receptors, Antigen, T-Cell