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T-cell nonmalignant clonal proliferation in ataxia telangiectasia: a
cytological, immunological, and molecular characterization
MH Stern, I Theodorou, A Aurias, M Maier-Redelsperger, M Debre, P Debre and C Griscelli
INSERM U132, Hopital Necker, Paris.
Cytogenetically abnormal T-cell nonmalignant clones are a characteristic
feature of ataxia telangiectasia (AT). Here, we study a t(14;14) clone from
a patient with AT, and provide a cytological, immunological, and molecular
characterization. This cellular population is clonal at the molecular
level, but is phenotypically heterogeneous, with CD4+CD8+ and CD4-CD8+
cells. Although these cells do not divide in the peripheral blood, a
majority of them are found in G1 phase and express the membrane antigen
4F2, a very early marker of activation. Many similarities are found between
this nonmalignant AT clone and T- cell prolymphocytic leukemia at the
morphologic, cytogenetic, and immunologic levels, despite the different
clinical courses associated with these proliferations. We hypothesize that
the t(14;14) translocation is linked to the abnormal morphology and
immunophenotype of the AT clone cells, but that this translocation confers
only a preactivated state to the cells. A complete malignant transformation
would then be due to secondary events.
Volume 73,
Issue 5,
pp. 1285-1290,
04/01/1989
Copyright © 1989 by The American Society of Hematology
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