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What
are Myelodysplastic Syndromes (MDS)?
Myelodysplastic Syndromes or MDS
is the name given to a group of similar blood
disorders in which a defect occurs in the stem
cells within the bone marrow. In a healthy individual,
stem cells produced in the bone marrow develop
into one of the three types of mature blood cells:
red blood cells which deliver oxygen throughout
the body; white blood cells which fight infection;
and platelets which help prevent bleeding. In
individuals with MDS, stem cells do not mature
into healthy blood cells. Instead, these immature
blood cells, called blasts, function improperly.
They either die in the bone marrow or soon after
they enter the blood. This results in the production
of too many defective blood cells and not enough
normal blood cells. In the United States there
are between 12,000 and 20,000 new cases of MDS
diagnosed each year, primarily among adults aged
60-80. |
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What
are the risk factors for MDS?
- Age – 60 years or older
- Heredity – having a family member
with certain forms of MDS
- Past treatment with chemotherapy or radiation
therapy
- Exposure to certain chemicals, including
tobacco smoke, pesticides, and other solvents
such as benzene.
What
are the signs/symptoms of MDS?
The symptoms of MDS vary depending on the individual
and the extent of the disease. Many patients
have no symptoms when they are initially diagnosed
because routine blood tests
can? detect MDS. Symptoms
may include the following:
- Shortness of breath
- Fatigue
- Skin that is paler than usual
- Easy bruising or bleeding
- Frequent infections
- Fever
- Weight loss
These symptoms could be the result of a number
of illnesses. Always consult your physician for
a proper diagnosis.
How
is MDS diagnosed?
In addition to a physical exam and complete
medical history, the following diagnostic tests
may be used:
- Complete Blood Count (CBC)—a simple
blood test which gives a profile of the components
of your blood (red blood cells, white blood
cells, platelets, and hemoglobin.)
- Peripheral Blood Smear—a sample of
blood is checked for changes in the number,
type, shape, and size of blood cells as well
as for the amount of iron in the red blood
cells.
- Bone Marrow Aspiration and/or Biopsy—a
needle is inserted into the hipbone or breastbone
to remove of a small piece of bone and/or bone
marrow for analysis.
- Cytogenetic Analysis—a sample of blood
or bone marrow cells are examined for certain
changes in the chromosomes.
What
are the different types of MDS?
There are several different types of MDS:
- Refractory anemia—This
type of MDS affects only the red blood cells.
Too few red blood cells cause the patient to
become anemic. The number of white blood cells
and platelets is normal.
- Refractory anemia with ringed sideroblasts—Similar
to refractory anemia, this type of MDS is characterized
by too few red blood cells. In addition, the
red blood cells have too much iron. The number
of white blood cells and platelets is normal.
- Refractory anemia with excess blasts—Again,
there are too few red blood cells in the blood
causing anemia. In addition, five percent to
19% of the cells in the bone marrow are blasts
(immature blood cells) and there are an abnormal
number of blasts found in the blood. Changes
to the white blood cells and platelets are
also possible. This type of MDS may progress
to acute myeloid leukemia if 20 % or more of
the cells in the bone marrow are blasts.
- Refractory cytopenia with multilineage
dysplasia—In this type of
MDS there are too few of at least two types
of blood cells. Also, less than five percent
of the cells in the bone marrow are blasts
(immature blood cells) and less than one
percent of the cells in the blood are blasts.
There may be an excess of iron in the red
blood cells as well. This type of MDS may
progress to acute leukemia.
- Myelodysplastic syndrome associated
with an islolated del(5q) chromosome abnormality—This
type of MDS is caused by a chromosomal deficiency.
Also, too few red blood cells cause anemia
and less than five percent of the cells in
the bone marrow and blood are blasts (immature
blood cells.) Once associated with a dire
prognosis, this type of MDS is now treatable
with a new medication called lenalidomide.
- Unclassified myelodysplastic syndrome—This
type of MDS is characterized by too few of
just one type of blood cell in the blood. Also,
the number of blasts (immature blood cells)
in the bone marrow and blood is normal, and
the disease is not one of the other forms of
MDS.
What
are the treatment options for patients with
MDS?
- Supportive Care—Maintaining
overall health and responding quickly to infections
are extremely important for patients with MDS.
Adequate amounts of certain vitamins and minerals,
such as vitamin B12 and folic acid, are necessary
to maximize the bone marrow’s ability
to produce red blood cells. Transfusions of
red blood cells or platelets are sometimes
needed to maintain adequate blood counts. Iron
levels are carefully monitored, especially
in MDS patients receiving regular red blood
cell transfusions, to avoid a potentially damaging
overload of iron in the body. New medications
which are effective in treating this iron overload
are now available.
- Growth Factors—Growth
factors are natural chemicals in the body that
cause the bone marrow to produce blood cells.
Recently, some growth factors have been manufactured
as drugs for patients with MDS. These drugs
are injected to stimulate the bone marrow to
increase its blood cell production. By increasing
blood counts these drugs can often reduce the
need for blood transfusions.
- Immunosuppressive Drug Therapy—With
some forms of MDS, the immune system may interfere
with the bone marrow’s ability to produce
normal blood cells. Immunosuppressive therapy
(drugs that suppress the immune system) has
been successful in treating patients with some
forms of MDS.
- Chemotherapy—Currently,
there are several drugs available for the treatment
of MDS. Both FDA-approved and experimental
(part of a clinical trial), these drugs affect
bone marrow cell growth and maturation. (more
info)
- Bone Marrow Transplants—At
this time, the only option for curing MDS is
a bone marrow transplant. In this procedure,
bone marrow is taken from a healthy donor and
given to the patient to replace the abnormal
marrow. Bone marrow transplants are most successful
in otherwise-healthy patients with marrow from
a matched donor, most commonly, from a sibling.
- Lenalidomide –This
is a new medication which is effective in treating
MDS associated with an isolated del(5q) chromosome
abnormality. It is just one example of a recent
advancement turning a dire prognosis into a
hopeful cure.
Huntington Hospital’s Don Monti
Cancer Center participates in clinical
trials for new drugs to treat patients with
MDS. Click
here for more information about clinical
trials.
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