Mom and Bessie 2013 |
Now they want to follow up with a bone marrow sample which is quite painful, but may give more answers. They are throwing around the word myelodysdplasia, which means......
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Myelodysplastic
syndrome (MDS) is a term for a collection of diseases that involves the
improper function of the bone marrow. It occurs when blood cells die in
the marrow, resulting in anemia and other issues surrounding the blood.
In many cases, people with MDS develop a form of leukemia
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html
OVERVIEW
The
myelodysplastic syndromes (MDS, myelodysplasia) are a group of blood
disorders associated with low blood counts. Normally, blood cells are
produced by the bone marrow (the spongy area in the middle of bones) in a
controlled fashion. In MDS, the bone marrow does not function
effectively to produce one or more types of blood cells, including red
blood cells, white blood cells, and platelets.Each type of blood cell performs a number of functions, including the following:
- Red blood cells carry oxygen throughout the body
- White blood cells help to protect the body from infection
- Platelets helps blood to clot normally
MDS may occur on its own (called de novo MDS) or 1 to 15 years after being exposed to certain forms of chemotherapy or radiation (called treatment-related MDS). Most people (approximately 75 percent) are older than 60 years when they are diagnosed with MDS, although it can develop during childhood.
SYMPTOMS
Some
people with MDS have no symptoms, and are diagnosed after laboratory
testing is done for another reason. Most people with MDS seek care due
to symptoms of anemia, including fatigue, weakness, becoming tired
quickly during activities, chest pain, dizziness, difficulty thinking
clearly, or shortness of breath.Less commonly, a person with MDS is diagnosed as a result of an infection, easy bruising, or bleeding. Symptoms such as fever and weight loss are uncommon early in the disease.
DIAGNOSIS
- A complete blood count indicates the number of red and white blood cells, and platelets
- A blood smear involves examining a small sample of blood under a microscope to examine the number, size, shape, and type of blood cells
- Cytogenetic analysis examines the blood or bone marrow cells for signs of genetic abnormalities in the chromosomes. Researchers have discovered that the genetic makeup of the cells can vary, which can affect how a particular patient responds to treatment. Interpretation of these studies is very complicated. In general, outcomes are better for patients with normal chromosomes and worse for patients with complex changes in their chromosomes. However, certain chromosomal changes are associated with even better outcomes that those seen with normal chromosomes. Examples of these good changes include loss of the Y chromosome or deletion of the long arm of chromosomes 5 or 20.
- Bone marrow aspiration and biopsy is performed to remove a sample of bone marrow from inside of a bone (usually the hip bone) and examine it with a microscope to look for abnormal cells.
TYPES OF MYELODYSPLASTIC SYNDROME
Perhaps the most useful clinical classification systems for MDS are the original and revised International Prognostic Scoring Systems (IPSS and IPSS-R, respectively). These models were devised to consider variables such as the percentage of immature blood cells (blasts) in the bone marrow, the type of blood abnormality present, as well as studies of the genetic makeup of the abnormal cells. Based on these criteria, the original IPSS defines four risk groups: low, intermediate-1, intermediate-2, and high-risk groups. Similar information is used by the IPSS-R to define five risk groups: very low, low, intermediate, high, and very high risk groups.
Treatment recommendations are based upon the patient's original or revised IPSS risk group; a person with low risk type MDS may live for many years before needing treatment while a person with high-risk type MDS usually needs more immediate treatment, without which his/her life expectancy may not exceed one to two years
TREATMENT
For patients with symptoms, our treatment approach is similar to that proposed by the National Comprehensive Cancer Network (NCCN). We choose a treatment based upon the patient's age, performance status (a measure of how well a patient can perform normal daily tasks), and disease characteristics such as the IPSS risk score.
Treatment options — Treatment options for patients with MDS typically fall into one of three categories:
- Supportive care includes the use of antibiotics for infection and transfusions for low blood counts. Supportive care is an important part of the management of all patients with MDS.
- High intensity treatment is more likely to be associated with “treatment-related mortality”, may require hospitalization, and includes combination chemotherapy with or without bone marrow transplantation. The trade-off for the higher risk associated with these therapies is a greater chance of effectiveness.
- Low intensity treatment includes those treatments less likely to produce treatment-related mortality and those that do not require a person to remain in the hospital, and includes use of hematopoietic growth factors, low intensity chemotherapy, immunosuppressive treatments, or a thalidomide derivative.
Treatment recommendations — Our general approach to the treatment of MDS is as follows:
- Patients with higher risk MDS (IPSS score greater than 1.5 or IPSS-R score greater than 4.5) who are young and otherwise healthy are generally treated with high intensity therapies (see 'High intensity treatments' below).
- Patients with lower risk MDS (IPSS score less than 1.5 or IPSS-R score less than 3) are generally treated with low intensity therapy or supportive care alone (see 'Low intensity treatments' below).
- Patients with intermediate risk MDS (IPSS-R score between 3 and 4.5) can be treated with either approach.
- Supportive care is an important adjunct to the management of all patients with MDS.
LOW INTENSITY TREATMENTS
Supportive treatments — Supportive
care includes treatment for the signs or symptoms of MDS, including a
low white blood cell, platelet, or red blood cell count. Due to the
advanced age of most patients with MDS and the chronic nature of the
disease, supportive care is an important part of treatment for all
patients. These treatments are not intended to cure the disease,
although they can improve a person's quality of life and may prolong
survival.
Blood transfusions — If
a person's red blood cell or platelet count becomes dangerously low, it
is possible to give donated blood. A person may donate whole blood or
single components, such as red blood cells or platelets. All donated
blood and blood products are tested for infectious diseases. Thus, the
risk of developing an infection as a result of transfused blood products
is now very low.
- Red blood cells — Transfusions of red blood cells may be needed to treat signs or symptoms of anemia, including feeling tired or short of breath. If frequent or multiple transfusions of red blood cells are needed, iron overload may occur, which might lead to organ damage. Although controversial, some investigators believe that concern about iron overload should not occur until the patient has received approximately 30 units of packed red blood cells. A treatment called iron chelation may be recommended to remove this excessive iron from the body. Iron chelation treatments can be taken by mouth or as an injection under the skin or into a vein. These treatments have relatively few side effects, although it is unclear if their use prolongs life or improves its quality.
- Platelets — Transfusions of platelets may be needed to prevent or treat bleeding problems caused by too few platelets.
Hematopoietic growth factors — Hematopoietic
growth factors are proteins that promote the growth and development of
blood cells. The use of growth factors may reduce a person's need for
blood transfusions. However, many people with MDS do not respond
normally to hematopoietic growth factors because of the bone marrow's
defective production of blood cells.
- Recombinant human granulocyte colony-stimulating factor (G-CSF, Neupogen) or recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF) stimulate white blood cell (granulocyte) production, and may raise the patient's white blood cell count. Use of G-CSF alone is not recommended, except perhaps in the setting of severe infection.
- Recombinant human erythropoietin (EPO, Procrit, Epogen) promotes the growth of red cells, and decreases the need for red cell transfusions in 20 percent of MDS patients.
Low intensity chemotherapy — Chemotherapy
medications in MDS aim to change bone marrow cells to develop more
normally, allowing for improved production of red cells, white cells,
and platelets. Low doses of a single chemotherapy drug may be
recommended for people with lower risk MDS or for people with
intermediate or high IPSS scores who cannot tolerate high intensity
chemotherapy or bone marrow transplantation.
- Azacitidine — Azacitidine (Vidaza) may increase survival and improve quality of life when compared with supportive treatments alone. Azacitidine is often given to patients with higher risk MDS. While a trial randomizing such patients to “best supportive care” (principally transfusions) or azacitidine found that survival was longer in the azacitidine group, the median improvement was only about six to nine months.
- Decitabine — Decitabine (Dacogen) is similar to azacitidine and appears to increase rates of complete remission. Complete remission means that there are no detectable blasts in the blood or bone marrow and that the bone marrow is functioning normally.
- Lenalidomide — Lenalidomide, a thalidomide-like drug, is particularly effective for people with anemia and lower risk MDS with abnormalities of chromosome 5 (called the 5q minus syndrome). Such patients may no longer require red blood cell transfusions after treatment with this agent.
Immunosuppressive drugs — In
some patients with MDS, the immune system causes the bone marrow to
slow production of blood cells. This may be especially true in people
with a reduced number of cells in the bone marrow (called marrow
hypoplasia).
Some of these patients, particularly those who are
younger, with early stage disease and a reduced cell content of the bone
marrow, respond to immunosuppressive therapies, which counter this
immune attack on the bone marrow, with a resulting increased effective
blood cell production. Use of an immunosuppressive therapy may allow
between 50 to 60 percent of people who have the HLA DR2 tissue type to
discontinue red blood cell transfusions.Examples of immunosuppressive therapies include antithymocyte globulin (ATG) and cyclosporine. ATG is usually given into a vein once per day for four days while cyclosporine is usually taken by mouth twice per day for as long as it is effective.
Most everyone who is treated with ATG develops serum sickness, which causes hives, swelling, and fever. This reaction can be minimized by giving steroid treatment along with the ATG.
HIGH INTENSITY TREATMENTS
High intensity chemotherapy — Patients
with intermediate or high risk type MDS may be treated with a
chemotherapy regimen similar to that used for treatment of acute myeloid
leukemia. In this group, chemotherapy is used to destroy abnormal cells
or prevent them from growing. However, this treatment is only
recommended if the person is relatively young .
High
intensity chemotherapy is not generally recommended for people with a
poor performance status, particularly if they are older than 65, or
patients age >75. In this group, the expected benefit (prolonged
survival) may not be worth the anticipated discomfort, hospitalization,
or risk of dying from the toxicity of chemotherapy. The exception would
be if the patient were placed on a trial of an investigational therapy
with benefits that might plausibly justify the risk.In some patients, supportive care can provide benefits that are equal to standard chemotherapy, with a lower risk of complications or toxicity. It should be stressed, however, that neither alternative is satisfactory prompting interest in clinical trials, many of which are ongoing. Some people do better with an approach that treats MDS-related problems, such as infection or anemia, as they occur, rather than trying to cure the disease. Transfusions and antibiotics can be given as needed in place of more aggressive forms of therapy.
Blood and bone marrow transplantation — Bone
marrow transplantation (also called hematopoietic stem cell
transplantation) is the only known treatment for MDS that has the
potential to induce long-term remission or cure. However,
transplantation involves the use of high intensity chemotherapy,
sometimes with whole body radiation, to eliminate all dividing cells in
the bone marrow. Unfortunately, the risks of treatment may be greater
than the benefits in some situations.
In the past, patients over
age 50 were not considered for bone marrow transplantation, mostly due
to the risk of transplant-related complications. Improvements have
allowed the upper age limit for such transplantation to expand to people
age 65 or more. However, approximately 75 percent of patients with MDS
are older than 60 at diagnosis, so conventional transplantation can only
be offered to a minority of individuals. For treatment of MDS, the optimal source of stem cells is a brother or sister with a similar genetic makeup (ie, a matched related donor). In general, parents, children, and relatives are not suitable donors, since they do not share the same parents and therefore do not have the same genetic material. Improvements in the ability to match unrelated donors has improved over the years and allowed for the transplantation of persons without a matched related donor. A donor's blood (peripheral blood stem cells) has largely replaced bone marrow the source of stem cells.
Use of reduced intensity chemotherapy treatment before transplantation may allow some patients with MDS, who would not otherwise be eligible, to undergo transplantation with a lower incidence of transplant-related complications. Reduced intensity regimens use less intensive chemotherapy with low dose or no radiation before transplantation with matched stem cells.
Transplantation is recommended for people with higher risk MDS who are under the age of 60 and who have a tissue-matched sibling donor, but not for people with lower risk disease. Although there is a significant chance of cure after bone marrow transplantation in low risk patients (approximately 60 percent), transplant-related deaths and the relapse rate at five years are also high (as high as 40 percent)
.
PROGNOSIS
We are quite worried as you can imagine, I guess there is nothing for us to do but wait for the bone marrow test.
Myelodysplasia is a
collection of conditions that result when bone marrow cells produce
damaged cells that don't mature properly. This can lead to low numbers
of red or white blood cells and platelets
Read more : http://www.ehow.com/facts_6952247_myelodysplasia.html
Read more : http://www.ehow.com/facts_6952247_myelodysplasia.html
Myelodysplastic
syndrome (MDS) is a term for a collection of diseases that involves the
improper function of the bone marrow. It occurs when blood cells die in
the marrow, resulting in anemia and other issues surrounding the blood.
In many cases, people with MDS develop a form of leukemia
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html
Myelodysplastic
syndrome (MDS) is a term for a collection of diseases that involves the
improper function of the bone marrow. It occurs when blood cells die in
the marrow, resulting in anemia and other issues surrounding the blood.
In many cases, people with MDS develop a form of leukemia
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html
Read more : http://www.ehow.com/facts_4967222_myelodysplasia-disease_.html