here is what the site says on drug treatment:
Understanding CMML
Incidence
Signs and Symptoms
Diagnosis
Treatment
Clinical Trials
Outcomes
Get More Information
Contact
Us
Understanding CMML
Chronic myelomonocytic leukemia (CMML) is an uncommon blood cancer that has
features of two other types of blood cancers. For this reason, the World Health
Organization (WHO) classified CMML as a "mixed myelodysplastic/
myeloproliferative disease." This relatively new classification (2001) is
expected to lead to a better understanding of the disease and to the development
of more effective treatments.
CMML begins with one or more changes (mutations) to the DNA of a type of
white cell called a "monocyte." When monocytes leave the blood and enter the
tissues, they attack invading organisms, help combat infection and assist other
blood cells, such as lymphocytes, in carrying out their immune functions.
Monocytes arise from immature blood-forming cells called "myeloblasts" and
"myelocytes."
In CMML, myeloblasts and myelocytes accumulate in the marrow and in other
organs, interfering with the normal production of monocytes and other types of
blood cells, including red blood cells and platelets. Monocytes represent about
5 to 10 percent of the cells in normal blood.
The World Health Organization categorized CMML into two subtypes according to
the percentage of blast cells (blasts) found in the blood and marrow
blasts in the marrow
the marrow
In most healthy individuals, blast cells represent less than 5 percent of
developing marrow cells.
Incidence
It is estimated that CMML affects approximately 3 out of 100,000 individuals
in the United States each year. The disease generally affects older adults.
Seventy-five percent of patients are older than 60 years at the time of
diagnosis and there are approximately twice as many male CMML patients as female
CMML patients.
Signs and Symptoms
Signs and symptoms may include
bleeding due to low platelet counts (thrombocytopenia)
Diagnosis
A CMML diagnosis generally cannot be confirmed after one lab test result
shows abnormal blood counts. The diagnosis can only be confirmed after a patient
has been monitored for a period of time with repeat lab tests to rule out other
forms of myelodysplastic syndromes (MDS) or myeloproliferative disorders
(MPDs).
The tests typically used in diagnosis include blood tests and bone marrow
aspiration and biopsy to check for
1,000/microliter of blood)
into red cells, certain types of white cells or platelets.
Other diagnostic tests may include
spleen or liver
or the BCR-ABL rearrangement gene. The Ph chromosome is an abnormality of
chromosome 22 found in the marrow and blood cells of CML patients
an enzyme found in saliva, tears and some immune cells such as monocytes
dehydrogenase" (LDH) and "beta 2-microglobulin." LDH levels may become elevated
when there is tissue damage in the body. Beta 2-microglobulin may increase due
to increased production or reduction of white cells, inflammation or certain
types of cancer.
Genetic Mutations. Twenty to 40 percent of CMML patients have
chromosomal abnormalities. About 1 to 4 percent of patients have an abnormality
known as a translocation involving the PDGFR-B and TEL genes. In a
translocation, a piece of one chromosome breaks off and attaches to another
chromosome, which can lead to the development of a cancer gene (oncogene).
Patients with the PDGFR-Band TEL gene mutation may respond
favorably to treatment with the drug imatinib (Gleevec®).
Treatment
For most CMML patients, the disease is treatable but not curable, with the
therapies currently available. Patients are advised to seek treatment from a
physician who is experienced in treating CMML or from a physician who is in
consultation with a center or physician who has experience treating the
disease.
The type of treatment depends on several patient factors, including the
Drug Therapy. There is no one standard treatment for CMML. Treatment
for previously untreated or relapsed patients may include standard-dose or low
dose cytarabine (Cytosar-U®), etoposide (VePesid®) and
hydroxyurea (Hydrea®). Treatment with these agents has been useful
for a small number of patients.
Azacitidine (Vidaza®) and decitabine (Dacogen®),
approved for treating MDS, are also approved
for treating CMML. However, the effectiveness of these two agents for CMML
treatment requires further study.
Imatinib (Gleevec®) is used to treat the small percentage of
patients who have the PDGFR-B and TEL gene mutation. This
treatment usually leads to a return to normal blood counts, cytogenetic
remissions and, occasionally, molecular remissions.
Stem Cell Transplantation. Allogeneic stem cell transplantation has
been used to treat and sometimes cure CMML patients. This option is available to
a small number of patients - generally younger patients who are not responding
to therapy and who have an appropriate stem cell donor.
For more information about stem cell transplantation, please see the free LLS
booklet Blood and
Marrow Stem Cell Transplantation.
Clinical Trials
The goal of clinical trials for CMML is to improve treatment and quality of
life and to increase survival. Patients are encouraged to talk to their
physicians about whether taking part in a clinical trials would be a good
treatment option for them. Patients can also call the Information Resource
Center (IRC) for more information about clinical trials for CMML.
Therapies currently under study for CMML treatment include
agents such as vorinostat (Zolinza®) and arsenic trioxide
(Trisenox®)
factor) - studies indicate that the fusion of DT388 (a toxin) with GM-CSF
allows the targeting of cells with GM-CSF receptors, such as CMML cells
patients who do not respond to drug therapy and are not eligible for allogeneic
stem cell transplants because of older age or other health risks.
Outcomes
Patients are advised to discuss survival information with their physicians.
The reported median survival of individuals diagnosed with CMML is 12 to 24
months after the start of treatment. Keep in mind that outcome data can show how
other people with CMML responded to treatment, but cannot predict how any one
person will respond. Also, statistics may underestimate survival to a small
degree since they may not reflect the most recent advances in treatment.
Many factors influence patient survival. Factors that may indicate a less
favorable outcome include
In about 20 percent of CMML patients, the disease progresses to acute
myelogenous leukemia (AML).
Get More information
For more information about CMML, read or order online the free LLS fact sheet Chronic
myelomonocytic leukemia (CMML) and juvenile myelomonocytic leukemia
(JMML)
