| |
|
|
|
|
|
|
|||
|
LM Wiedemann, KK Karhi, MK Shivji, SI Rayter, SM Pegram, G Dowden, D Bevan, A Will, DA Galton and LC Chan
Leukaemia Research Fund Centre, Institute of Cancer Research, Chester
Beatty Laboratories, London, UK.
The chromosome 22 derivative, the Philadelphia (Ph) chromosome, results
from a reciprocal translocation t(9;22) (q34;q11) and is associated with
chronic myeloid leukemia (CML). The translocation can be identified at the
DNA level in Ph-positive CML by using a probe to the breakpoint cluster
region (bcr). In addition, as a result of this translocation an abl-related
210-kd protein with protein tyrosine kinase (PTK) activity is produced. We
analyzed 28 cases of Ph-negative CML for rearrangement of the chromosome 22
sequences and found that eight of the 28 show rearrangement of the bcr.
When 12 of the Ph- negative cases were independently reviewed, five were
indistinguishable from Ph-positive CML on the basis of morphology,
peripheral blood film and clinical details. These five also showed bcr
rearrangement. The other seven were reclassified as six atypical CML (aCML)
and one chronic myelomonocytic leukemia (CMML). None of these seven showed
bcr rearrangement. In addition 11 cases of bcr- CML were assayed for abl-
related PTK, and no detectable activity was present, whereas p210 phl/abl
PTK was observed both in Ph-positive (three cases examined) and
Ph-negative, bcr + (four cases examined) CML. Therefore, bcr + CML, whether
or not the Ph chromosome is cytogenetically apparent, involves a similar
molecular alteration and produces the 210-kd protein with enhanced PTK
activity. Furthermore, these cases can be distinguished from Ph-negative
bcr- CML by careful evaluation of clinical and hematologic data.
This article has been cited by other articles:
| |||||||||||
| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||