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Myelodsysplastic
Syndrome
Myelodysplastic syndromes are a
group of diseases characterized by the body's
inability to produce healthy blood cells.
Myelodysplasia is sometimes referred to
as pre-leukemia because myelodysplastic
syndromes begin in the bone marrow stem
cells and eventually progress to acute myelogenous
leukemia. The myelodysplastic syndromes
are really several different diseases, each
with a different prognosis.
Myelodysplastic
Syndrome can be caused by chemical exposures
in the workplace from solvents such as benzene.
These are often found in petrochemical industry
and plastics industry among others.
Prior to 1980, many products such paints,
paint thinners, varnishes, degreasers, amd
industrial solvents contained benzene.
Some myelodysplastic syndromes develop after
treatment with chemotherapy or radiation
therapy for cancer and are referred to as
secondary myelodysplasia.
Currently, an allogeneic stem cell transplant
is the only curative therapy; however, patients
may benefit from other types of treatment.
Following
is a description of four classifications:
Refractory
Anemia (RA): Patients have low
blood counts, bone marrow blasts are less
than 5%, and sideroblasts (iron containing
cells) are less than 15%. The average survival
is approximately 43 months, but can be influenced
by specific chromosomal abnormalities.
Refractory
Anemia with Ringed Sideroblasts (RARS):
Patients have low blood counts, bone marrow
blasts are less than 5% and sideroblasts
are greater than 15%. The average survival
is 55 months, but can be influenced by specific
chromosomal abnormalities.
Refractory
Anemia with Excess Blasts (RAEB):
Patients have low blood counts, 1-5% blasts
in the blood and bone marrow blasts between
5 and 20%. The average survival is 12 months,
but can be influenced by specific chromosomal
abnormalities.
Refractory
Anemia with Excess Blasts in Transition
(RAEBt): Patients have low blood
counts, over 5% blasts in the blood or cells
in the blood containing anabnormality referred
to as Auer rods and bone marrow blasts between
20 and 30%. The average survival is 5 months,
but can be influenced by specific chromosomal
abnormalities.
Treatment
of myelodysplastic syndromes is
individualized and depends on two main factors:
the severity of low blood counts and the
risk of progression to acute myeloid leukemia.
The goals of treatment are to manage the
complications of ineffective blood cell
production and attempt to cure the disease
when possible. A patient's age, other medical
conditions and the severity of the myelodysplastic
syndrome all influence treatment decisions.
The objective of treatment is to control
the growth of the abnormalmyelodysplastic
cells so that the more normal cells can
grow and improve blood cell production.
The goal of allogeneic stem cell transplant
or other aggressive treatment approaches
is to cure patients by completely eliminating
all the myelodysplastic cells. Allogeneic
stem cell transplants produce the best results
in younger individuals. Patients unable
or unwilling to undergo an allogeneic stem
cell transplant can be treated with chemotherapy,
biologic therapy or investigational anti-cancer
agents on clinical trials.
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