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

Abstract

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.

Immunophenotypic and clinical features of T‐cell receptor γδ+ T‐lineage acute lymphoblastic leukaemia

“…Our results in five children with TCR‐γδ + CD3 + CD4 − CD8 − T‐ALL could not confirm the particular clinical features of this subgroup (e.g. very young age at diagnosis, high leucocyte counts, discrete lymph node enlargements) which have been previously reported by Alfsen et al (1991 ). The worse prognosis of children with TCR‐γδ + CD3 + CD4 − CD8 − T‐ALL, suggested by Alfsen et al (1991 ), was also observed in our series, since three of five children relapsed within the first year of treatment and two of them died a few weeks afterwards.…”

“…Some recent studies have suggested that TCR‐γδ + T‐ALL represent an important subgroup of T‐lineage ALL with distinctive clinicopathologic features. These studies, however, were based on a small number of patients ( Gouttefangeas et al , 1990 ; Alfsen et al , 1991 ) and immunological marker analyses not suitable for the reliable distinction of TCR‐αβ + and ‐γδ + T‐ALL ( Macintyre et al , 1990 ). Our results in five children with TCR‐γδ + CD3 + CD4 − CD8 − T‐ALL could not confirm the particular clinical features of this subgroup (e.g.…”

“…Recent studies in T‐cell malignancies have suggested that TCR expression might contribute to a biologically based understanding of clinicopathologic features and prognosis of T‐ALL patient subgroups ( Macintyre et al , 1990 ; Alfsen et al , 1991 ; Campana et al , 1993 ), and identification of T‐cell lymphoma subgroups (i.e. hepatosplenic γ/δ lymphoma) with characteristic morphologic appearance and clinical behaviour ( Jaffe, 1996).…”

Molecular cytogenetics of childhood hematological malignancies

“…Here, the major subgroups of T-ALL are distinguished based on the membrane expression of TCR-␣/␤ chains (group a) and TCR-␥/␦ chains (group b). 132 While several authors agreed to separate these two clinically and phenotypically distinct T-ALL subtypes, [132][133][134] others remain reluctant. 135,136 In that respect, Schott et al 137 reported a better outcome for patients with T cell ALL with TCR-␥/␦ expression.…”