CHRONIC MYELOID LEUKEMIA

Chronic Myelogenous Leukemia (CML)

(Chronic Granulocytic Leukemia;

Chronic Myelocytic Leukemia; Chronic

Myeloid Leukemia)

Introduction
Chronic myelogenous leukemia (CML) occurs when a

pluripotent stem cell undergoes malignant transformation and

clonal myeloproliferation, leading to a striking overproduction
of immature granulocytes.
Initially asymptomatic, CML progression is insidious, with a
nonspecific benign stage (malaise, anorexia, weight loss)
eventually giving way to accelerated or blast phases with more
ominous signs, such as splenomegaly, pallor, easy bruising and
bleeding, fever, lymphadenopathy, and skin changes.
Peripheral blood smear, bone marrow aspirate, and
demonstration of Philadelphia chromosome are diagnostic.
Treatment is withimatinib, which significantly improves
response and prolongs survival.
The curative potential ofimatinibis undefined.
Myelosuppressive drugs (eg,hydroxyurea), stem cell
transplantation, and interferon alfa are also used.

Epidemiology
CML accounts for about 15% of all adult leukemias. CML

can strike at any age, although it is uncommon before

age 10, and the median age at diagnosis is 45 to 55.
CML may occur in either sex.
The American Cancer Society estimates that in 2014,
about 5,980 new cases of chronic myelogenous leukemia
were diagnosed, and about 810 people died of the
disease. This means that a little over 10% of all newly
diagnosed leukemia cases will be chronic myelogenous
leukemia. The average risk of a person getting this
disease is 1 in 588. The disease is more common in men
than women, and more common in whites than AfricanAmericans. The average age at diagnosis is 64 years,
and this disease is rarely seen in children

Symptoms and Signs

Patients are often asymptomatic early on, with

insidious onset of nonspecific symptoms (eg,

fatigue, weakness, anorexia, weight loss, fever,
night sweats, a sense of abdominal fullness), which
may prompt evaluation. Initially, pallor, bleeding,
easy bruising, and lymphadenopathy are unusual,
but moderate or occasionally extreme
splenomegaly is common (60 to 70% of cases).
With disease progression, splenomegaly may
increase, and pallor and bleeding occur. Fever,
marked lymphadenopathy, and maculopapular
skin involvement are ominous developments.

Peripheral
Blood
Smear in
ChronicPhase
Chronic
Myelogenou
s Leukemia

In chronic-phase chronic myelogenous

leukemia, the peripheral blood smear is

characterized by a constellation of
abnormalities including a striking
leukocytosis with pronounced
neutrophilia and basophilia, and,
frequently, eosinophilia. Although
myeloid elements in all stages of
maturation are present, blasts account
for < 5% of all leukocytes. Most patients
have thrombocytosis, and platelets are
often enlarged and pleomorphic.

Prognosis
Withimatinib, survival is>90% at 5 yr after

diagnosis for chronic phase CML.

Beforeimatinibwas used, with treatment, 5 to 10%
of patients died within 2 yr of diagnosis; 10 to 15%
died each year thereafter. Median survival was 4 to
7 yr. Most (90%) deaths followed a blast phase or an
accelerated phase of the disease. Median survival
after blast crisis was about 3 to 6 mo or longer if
remission was achieved.
Ph chromosomenegative CML and chronic
myelomonocytic leukemia have a worse prognosis
than Ph chromosomepositive CML. Their clinical
behaviors resemble a myelodysplastic syndrome