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Chronic Granulocytic Leukemia
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CML or chronic
myelogenous leukemia
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- Chronic Myelogenous Leukemia (CML) refers to an overproduction of
white blood cells (WBC) in the bone marrow. The marrow is
the site in the body where red blood cells (i.e., RBC, carry
oxygen to the cells of the body), white blood cells (i.e.,
WBC, fight infections), and platelets (form blood clots) are
formed.
- When excess white blood cells invade
the bone marrow, they also spill over into the blood. Since
they consist mostly of mature granulocytes, like
neutrophiles and myelocytes, they are able to function
normally. As a result, symptoms are less severe compared to
acute forms of leukemia, and develop more slowly.
- This condition has three phases,
which define symptom severity:
- Chronic phase, in which there are only a small number
of blast cells -- i.e., immature WBCs not capable of
fighting infection-in the blood or bone marrow, and thus
no signs or symptoms of leukemia.
- Acute or accelerated phase, with more blasts cells
found -- and more symptoms.
- Blastic phase, with 30% of the blood cells found to be
immature blast cells. Symptoms may be severe. Most cases
are seen in adults, but children also develop
CML.
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- Chronic phase may last for years
before entering the accelerated phase.
- Feeling tired
- Feeling discomfort in the left upper abdomen (due to
the enlargement of the spleen or splenomegaly)
- Mild shortness of breath
- Low grade fever
- Mild paleness
- Weight loss
- Loss of appetite
- Excessive sweating, especially at night
- Inability to tolerate warm temperature
- Mild bone pain
- Right upper abdominal fullness (enlarged liver
or hepatomegaly)
- Accelerated phase is actually a
transitional phase that may last for months to years, with
the above symptoms becoming more pronounced.
- More pronounced left upper abdominal pain
(splenomegaly)
- Bone pain
- Fever
- Paleness
- Shortness of breath
- Lack of energy and appetite
- Night sweats
- Bleeding -- skin Bruising, petechiae
(small areas of bleeding under the skin), and fever due to
infections. Patients appear very sick.
- Blast cells can also invade the
brain and the skin.
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- Unknown
- Damaged genetic material (DNA)
maybe responsible
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- Often patients unknowingly in the
chronic phase with mild or no complaints, will incidentally
have a physical exam or a blood test that reveals
splenomegaly, hepatomegaly, or increased numbers of WBC.
- During the accelerated or blastic
phases, patients may be admitted to the hospital with
complaints of infection, severe anemia, or bleeding.
- Blood tests:
- May show presence of (and percentage) blast cells
- Show increased numbers of WBC (mature granulocytes,
normal lymphocytes, basophiles, and eosinophils)
- May show mild to moderate anemia
- Platelet count may vary from low to normal to high
- In CML, the mature cells have absent (or low) levels of leukocyte Alkaline phosphatase (an enzyme normally present in
these cells).
- There may be an elevation of Uric acid in the
blood.
- Bone marrow content is often
examined under a microscope in order to identify leukemia
type and extent of the diseases. In blast crisis and the
accelerated stage, the marrow shows large numbers of WBCs
(blast cells).
- Cytogenetic testing on the cells
from the bone marrow will show the Philadelphia chromosome.
- The Polymerase Chain Reaction Test
(PCR) is a sensitive and accurate way of diagnosing CML.
- Imaging techniques of the abdomen (MRI, CAT scan, Ultrasound) show
enlargement of the spleen and
liver.
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- Ionizing radiation
- Presence of Philadelphia chromosome
-- an abnormal chromosome (number 22 contains the BCR gene)
found in the cells of CML patients Chromosome number 9 (ABL
gene) is also abnormal in CML patients. Abnormal fusion of
BCR-ABL leads to formation of an abnormal protein that may
be responsible for CML.
- Benzene.
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- Myelosuppression is the main form of therapy, intended
to reduce the number of white blood cells and restore the
count to normal levels.
- Medications such as Busulfan,
Hydroxyurea, Cytarabine, and Alpha Interferon are some of
the medications most commonly used.
- Leukapheresis is a technique used
to filter the blood of excess WBCs.
- Bone marrow transplantation is often considered for
younger patients with a sibling (whose bone marrow closely
matches the patient's).
- Removal of the spleen is sometimes done if it is
enlarged.
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Contact
your physician as soon as possible. Be sure to ask about any
new treatments, research, clinical trials, or procedures
that may be available for
CML.
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