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JD Norton, J Pattinson, AV Hoffbrand, H Jani, JC Yaxley and BF Leber
Department of Haematology, Royal Free Hospital School of Medicine, London,
UK.
Fifty-nine patients with B cell chronic lymphocytic leukemia (B-CLL) were
screened for clonal rearrangement of T cell receptor (TCR) beta and gamma
chain genes. Four were found with rearranged TCR beta genes, but none had
detectable rearrangement of TCR gamma genes. One typical patient with B-CLL
had a TCR beta gene structure consistent with a variable-diversity-joining
rearrangement into the C beta 2 gene on one allele. An apparently identical
rearrangement pattern was seen in a second patient, which suggested that
there may be a restriction on the repertoire of possible TCR beta gene
recombinations in mature B cells. Two further patients had a simple
deletion of sequences, consistent with a diversity-joining rearrangement
into C beta 2 on one allele. All four patients had rearrangements of
immunoglobulin heavy- and light- chain genes typical of mature B cell
malignancies. However, on review of clinical, morphological, and
immunophenotype data, two had features consistent with B cell
prolymphocytic leukemia or B lymphoma, and a third had progressed to a
prolymphocytic transformation. Low-level expression of a predominantly 1.0-
to 1.2-kilobase germ line TCR beta gene transcript was detected in several
B-CLLs and at a comparable level in the four with rearranged TCR beta
genes. This, together with the low frequency of TCR gene rearrangement,
suggests that most B-CLL cases arise at a developmental stage when factors
required for TCR gene activity are not operative.
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| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||