Chronic Myeloid Leukemia (CML), a myeloproliferative disease of stem cell o
rigin, is characterized by the presence of the Philadelphia (Ph) chromosome
and the ber-abl oncogene. The BCR-ABL fusion gene product, thought to be c
ausative in CML, has multiple effects on diverse cell functions such as gro
wth, differentiation and turnover as well as adhesion and apoptosis. Persis
tent Ph-negative progenitors co-exist with leukemic cells, both in the marr
ow and blood of patients, in the early chronic phase or the disease. Despit
e accumulating knowledge or hemopoiesis and the disease process, CML remain
s incurable with conventional chemotherapy. Nonetheless, with the efficacy
of the ABL tyrosine kinase inhibitor STI-571 (signal transduction inhibitor
571) as a novel therapy in CML recently being realized in clinical trials,
it is therefore timely to review our current understanding of the cell bio
logy of this fascinating disease. Copyright (C) 2001 John Wiley & Sons, Ltd
.