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Clonality in juvenile chronic myelogenous leukemia
L Busque, DG Gilliland, JT Prchal, CA Sieff, HJ Weinstein, JM Sokol, M Belickova, AS Wayne, KS Zuckerman and L Sokol
Division of Hematology-Oncology, Brigham and Women's Hospital, Harvard
Medical School, Boston, MA.
Juvenile chronic myelogenous leukemia (JCML) is a myeloproliferative
disease in which morbidity and mortality are primarily caused by
nonhematopoietic organ failure from myelomonocytic infiltration or by
failure of the normal bone marrow. Morphologic evidence of maturation
arrest, karyotypic abnormalities, and progression to blast crisis are
infrequent events. Viral infections and other reactive processes can
initially mimic the clinical course of JCML, creating diagnostic problems.
Because of the rarity of JCML and technical limitations, formal clonality
studies have not been reported previously. Nine female JCML patients were
identified by clinical criteria, characteristic 'spontaneous' in vitro cell
growth, and negative cultures and titers for various viral agents.
Peripheral blood and bone marrow samples were obtained at the time of
diagnosis for cell separation and RNA and DNA isolation. To assess
clonality, X-chromosome inactivation patterns were evaluated using three
different, recently developed polymerase chain reaction-based clonality
assays. All nine female JCML patients showed evidence for monoclonal origin
of mononuclear cells at the time of diagnosis. Cell separation studies
further traced the monoclonal origin back to at least the most primitive
myeloid progenitor cell. Reversion to a polyclonal state was demonstrated
after bone marrow transplant and also in one patient following treatment
with 13-cis retinoic acid. This demonstration of clonality in JCML
delineates it from the reactive processes and provides a basis for
molecular genetic strategies to identify causally associated mutations.
Volume 85,
Issue 1,
pp. 21-30,
01/01/1995
Copyright © 1995 by The American Society of Hematology
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