Development of T-cell leukaemia in an ataxia telangiectasia patient following clonal selection in t(X;14)-containing lymphocytes.
Ataxia telangiectasia is a rare inherited and progressive neurological disorder in which patients show an unusual predisposition to T-cell leukaemia. We report here observations on a patient with a large cytogenetically abnormal clone showing a single t(X;14)(q28;q11) translocation which conferred a proliferative advantage on the cells. The further evolution of this clone to cytogenetically more complex clones of lymphocytes was seen in the patient. She subsequently developed a rapidly progressing T-cell leukaemia, with a CD4+CD8+ T-cell phenotype, about five years after the first appearance of additional chromosome translocations in the clone cells.
Taylor
A M
AM
Department of Cancer Studies, Medical School, University of Birmingham, UK.
Lowe
P A
PA
Stacey
M
M
Thick
J
J
Campbell
L
L
Beatty
D
D
Biggs
P
P
Formstone
C J
CJ
D001260:Ataxia Telangiectasia / Q000150:complications*; D002883:Chromosomes, Human, Pair 14; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D015458:Leukemia, T-Cell / Q000150:complications / Q000235:genetics* / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology*; D014178:Translocation, Genetic; D014960:X Chromosome