Chronic Myelogenous
Leukemia (CML), also known as chronic myeloid or
chronic myelocytic leukemia, is a malignant cancer
of the myeloid line of cells in the bone marrow that
results in the uncontrolled growth of white blood
cells in the bone marrow, blood, and body tissues.
It is estimated that about 5,000 people in the
United States are diagnosed with CML each year. CML
affects males more than females and usually occurs
in middle age adults. Although children can also
develop the disease, childhood cases of CML account
for only about 2% of all people who develop the
condition.
The exact cause of CML is not
known but research over the past 40 years has
expanded our understanding of the genetic and
molecular events that lead to the development of
CML. The major genetic abnormality that is observed
in the marrow cells of more than 90% of patients
with CML is the Philadelphia (Ph) chromosome.
Many
patients in the early stages of CML have no symptoms
while others may have non-specific symptoms such as
generalized fatigue, fever, night sweats, or an
enlarged spleen. In many cases, CML is diagnosed
during a routine blood test which shows an abnormal
increase in white blood cells (leukocytosis). The
hallmark for diagnosis of CML is demonstrating the
presence of the Philadelphia (Ph) chromosome in bone
marrow cells.
Three clinical stages of CML
are recognized which are important in terms of
predicting the prognosis (outlook) as well as
planning treatment for the disease. The three
clinical stages of CML include the chronic-phase;
the accelerated-phase; and the blastic-phase.
Approximately 80% of patients with CML are diagnosed
in the chronic-phase of the disease which can last
from a few months to about 4-5 years. The
chronic-phase can usually be controlled with
medications.
Nearly 80% of patients with CML will
progress from the chronic-phase to the
accelerated-phase of the disease which is a more
advanced stage of the disease. Clinical symptoms of
CML as well as blood abnormalities become more
prominent during the accelerated-phase of CML. The
third stage of CML is known as the blastic-phase and
is the most advanced stage of the disease. It is
called the "blastic-phase" because 30% or
more of immature, leukemic white blood cells called
"blasts" are found in the bone marrow and
blood. When clinical symptoms of CML occur in the
blastic-phase of the disease, this is known as "blast
crisis".
The treatment options for
patients with CML depend to a large extent upon the
patient's age, stage of the disease, and overall
health. In general, the treatment options for CML
can be divided into drug therapy and stem-cell
transplantation based therapy. Currently, the only
known treatment which is potentially curative for
CML is allogeneic stem cell transplantation. A
variety of different drugs is used for the treatment
of CML and can usually control the disease,
especially in the early chronic-phase of CML but
they do not cure the disease. Imatinib mesylate
(Gleevec), a drug that was approved by the FDA in
2001 for the treatment of CML, has had a dramatic
impact on the treatment of CML and has improved the
prognosis for many patients.
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