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High prevalence of T-cell receptor V delta 2-(D)-D delta 3 or D delta 1/2-D
delta 3 rearrangements in B-precursor acute lymphoblastic leukemias
A Biondi, P Francia di Celle, V Rossi, G Casorati, G Matullo, G Giudici, R Foa and N Migone
Clinica Pediatrica Universita di Milano, Monza, Italy.
Rearrangement of the immunoglobulin (Ig) and T-cell receptor (TcR) genes
generally has been considered a useful marker of B- and T-cell lineage in
lymphoproliferative disorders. However, concomitant rearrangements of Ig
and TcR genes have been commonly reported in the most immature lymphoid
malignancies, mainly in B-cell precursor acute lymphoblastic leukemia
(ALL). To better characterize the nature of this lineage promiscuity, we
have analyzed the configuration of the TcR delta locus in 75 B-precursor
ALL. The large majority of cases (87%) displayed a rearrangement or
deletion at the delta locus. Among the 57 nondeletional rearrangements, two
patterns were predominant and both appeared to derive from partial joining:
a V delta-(D)-D delta 3 (32/57) and a D delta 1/2-D delta 3 (11/57) type. A
single V delta gene (V delta 2) appeared to be involved in the first type
of rearrangement. These findings are at variance with T-ALL, where
rearrangements 5' to V delta 2 are usually found. It remains to be
elucidated whether this incomplete attempt of V delta 2 gene assembly is
related to the neoplastic event or represents a physiologic predisposition
occurring during early stages of the normal lymphocyte differentiation.
Volume 75,
Issue 9,
pp. 1834-1840,
05/01/1990
Copyright © 1990 by The American Society of Hematology
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