RAZELLE KURZROCK, M.D.; MARK B. BLICK, D.O.; MOSHE TALPAZ, M.D.; WILLIAM S. VELASQUEZ, M.D.; JOSE M. TRUJILLO, M.D.; NICOLA M. KOUTTAB, Ph.D.; WILLIAM S. KLOETZER, Ph.D.; RALPH B. ARLINGHAUS, Ph.D.; JORDAN U. GUTTERMAN, M.D.
We have followed one patient with Philadelphia (Ph)-negative chronic myelogenous leukemia and identified an additional four patients from the literature who showed the rearrangement in the breakpoint cluster region (bcr) on chromosome 22 characteristic of Ph-positive chronic myelogenous leukemia. The clinical course of these five patients was similar to that of Ph-positive patients, with easily controlled leukocyte counts, a prolonged benign phase, and prolonged survival. Furthermore, we have shown, for the first time, that bcr rearrangement in Ph-negative chronic myelogenous leukemia can result in expression of the aberrant 210-kilodalton bcr-abl fusion protein, which has been strongly implicated in Ph-positive leukemogenesis. Research data pertaining to possible cytogenetic mechanisms leading to production of p210bcr-abl in the absence of the Ph chromosome are reviewed. Molecular analysis provides an important tool for classifying and predicting prognosis of some patients with Ph-negative chronic myelogenous leukemia.
KURZROCK R, BLICK MB, TALPAZ M, et al. Rearrangement in the Breakpoint Cluster Region and the Clinical Course in Philadelphia-Negative Chronic Myelogenous Leukemia. Ann Intern Med. 1986;105:673–679. doi: 10.7326/0003-4819-105-5-673
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Published: Ann Intern Med. 1986;105(5):673-679.
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