Jane Doe
What is myelodysplastic syndrome?
Myelodysplastic syndrome (MDS) is the name of a group of conditions that occur when the blood-forming cells in the bone marrow are damaged. This damage leads to low numbers of one or more type of blood cells.
Also called preleukemia, myelodysplastic syndrome is a group of conditions in which blood cells do not reach a mature state, staying in the bone marrow. When these defective cells outnumber healthy cells, multiple problems like anemia, rapid bleeding or severe infection can develop.
Normal bone marrow
Bone marrow is found inside certain bones, including the skull, ribs, pelvis, and spine. It is made up of blood-forming cells, fat cells, and supporting tissues that help the blood-forming cells grow. A small fraction of the blood-forming cells are a special type of cell known as blood stem cells. Stem cells are needed to make new cells. When a stem cell divides it makes 2 cells: one cell that stays a stem cell, and another cell that can keep changing and dividing to make blood cells. There are 3 types of blood cells: red blood cells, white blood cells, and platelets.
Red blood cells pick up oxygen in the lungs and carry it to the rest of the body. These cells also bring carbon dioxide back to the lungs. Having too few red blood cells is called anemia. It can make people feel tired and weak and look pale. Severe anemia can cause shortness of breath.
White blood cells (also known as leukocytes) are important in defending the body against infection. The 2 major types of white blood cells are lymphocytes and granulocytes.
In about one-third of patients, MDS can progress to a rapidly growing cancer of bone marrow cells called acute myeloid leukemia. Because most patients do not get leukemia, MDS was previously classified as a disease of low malignant potential. Now that doctors have learned more about MDS, it is considered to be a form of cancer. The major reason is that MDS is a clonal disease, which means that there is a large population of abnormal cells that all came from a single, abnormal cell. These abnormal cells have the same genes -- just like identical twins -- and they share abnormal growth properties. Clonal growth is typical of cancer.
In the past, MDS was referred to as pre-leukemia and smoldering leukemia. Since most MDS patients do not get leukemia, these terms are not accurate and are no longer used.
Types of myelodysplastic syndrome
Symptoms of Myelodysplastic Syndrome
When a patient is first diagnosed with MDS their symptoms aren’t always apparent or easily diagnosed. Since everyone experiences this type of illness differently, some patients don’t even show signs or obvious symptoms of being ill right away. However, for patients who do show symptoms, these can usually relate to an abnormal blood count, which means that they have a low amount of red blood cells, white blood cells or platelets in their body.
For a person’s body to be in healthy state and free of illness like MDS, they must have the following:
Causes of Myelodysplastic Syndrome
Some cases of myelodysplastic syndrome (MDS) are linked to known risk factors, but for most, the cause is unknown
Some genes (parts of DNA) contain instructions for controlling a cell's growth and division process. Certain genes that promote cell division are called oncogenes. Other genes called tumor suppressor genes can slow down cell division or even cause cells to die at an appropriate time. Cancers can be caused by DNA mutations (gene defects) that turn on oncogenes or turn off tumor suppressor genes.
Previous Cancer Treatment - Research has proven that patients, who have received prior cancer treatment, whether through radiation or chemotherapy, are at higher risk of developing MDS. People who endure stem cell transplants are also more at risk
Exposure to Benzene - Exposure to large amounts of harmful chemicals, such as benzene, can cause myelodysplastic syndromes. Benzene is an industrial chemical that’s found in gasoline, detergents, cigarette smoke and can even contaminate well water.
Supportive Care
Lifestyle changes after Myelodysplastic Syndrome
You can't change the fact that you have had cancer. What you can change is how you live the rest of your life -- making choices to help you stay healthy and feel as well as you can. This can be a time to look at your life in new ways. Maybe you are thinking about how to improve your health over the long term. Some people even start during cancer treatment.
Making healthier choices
For many people, a diagnosis of cancer helps them focus on their health in ways they may not have thought much about in the past. Are there things you could do that might make you healthier? Maybe you could try to eat better or get more exercise. Maybe you could cut down on the alcohol, or give up tobacco. Even things like keeping your stress level under control may help. Now is a good time to think about making changes that can have positive effects for the rest of your life. You will feel better and you will also be healthier.
Eating better
If treatment caused weight changes or eating or taste problems, do the best you can and keep in mind that these problems usually get better over time. You may find it helps to eat small portions every 2 to 3 hours until you feel better. You may also want to ask your cancer team about seeing a dietitian, an expert in nutrition who can give you ideas on how to deal with these treatment side effects.
One of the best things you can do after cancer treatment is put healthy eating habits into place. You may be surprised at the long-term benefits of some simple changes, like increasing the variety of healthy foods you eat. Getting to and staying at a healthy weight, eating a healthy diet, and limiting your alcohol intake may lower your risk for a number of types of cancer, as well as having many other health benefits.
Rest, fatigue, and exercise
If you were sick and not very active during treatment, it is normal for your fitness, endurance, and muscle strength to decline. Any plan for physical activity should fit your own situation. An older person who has never exercised will not be able to take on the same amount of exercise as a 20-year-old who plays tennis twice a week. If you haven't exercised in a few years, you will have to start slowly – maybe just by taking short walks.
If you are very tired, you will need to balance activity with rest. It is OK to rest when you need to. Sometimes it's really hard for people to allow themselves to rest when they are used to working all day or taking care of a household, but this is not the time to push yourself too hard. Listen to your body and rest when you need to. (For more information on dealing with fatigue, please see Fatigue in People With Cancer and Anemia in People With Cancer.)
Keep in mind exercise can improve your physical and emotional health.
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Myelodysplastic syndrome (MDS) is the name of a group of conditions that occur when the blood-forming cells in the bone marrow are damaged. This damage leads to low numbers of one or more type of blood cells.
Also called preleukemia, myelodysplastic syndrome is a group of conditions in which blood cells do not reach a mature state, staying in the bone marrow. When these defective cells outnumber healthy cells, multiple problems like anemia, rapid bleeding or severe infection can develop.
Normal bone marrow
Bone marrow is found inside certain bones, including the skull, ribs, pelvis, and spine. It is made up of blood-forming cells, fat cells, and supporting tissues that help the blood-forming cells grow. A small fraction of the blood-forming cells are a special type of cell known as blood stem cells. Stem cells are needed to make new cells. When a stem cell divides it makes 2 cells: one cell that stays a stem cell, and another cell that can keep changing and dividing to make blood cells. There are 3 types of blood cells: red blood cells, white blood cells, and platelets.
Red blood cells pick up oxygen in the lungs and carry it to the rest of the body. These cells also bring carbon dioxide back to the lungs. Having too few red blood cells is called anemia. It can make people feel tired and weak and look pale. Severe anemia can cause shortness of breath.
White blood cells (also known as leukocytes) are important in defending the body against infection. The 2 major types of white blood cells are lymphocytes and granulocytes.
In about one-third of patients, MDS can progress to a rapidly growing cancer of bone marrow cells called acute myeloid leukemia. Because most patients do not get leukemia, MDS was previously classified as a disease of low malignant potential. Now that doctors have learned more about MDS, it is considered to be a form of cancer. The major reason is that MDS is a clonal disease, which means that there is a large population of abnormal cells that all came from a single, abnormal cell. These abnormal cells have the same genes -- just like identical twins -- and they share abnormal growth properties. Clonal growth is typical of cancer.
In the past, MDS was referred to as pre-leukemia and smoldering leukemia. Since most MDS patients do not get leukemia, these terms are not accurate and are no longer used.
The original classification of myelodysplastic syndrome (MDS) was developed more than 20 years ago at an international conference attended mostly by doctors from France, the United States, and Great Britain. This system was known as the French-American-British (FAB) classification.
The system used today is the World Health Organization (WHO) classification. This system seems to be more helpful than the FAB classification in predicting prognosis (outlook). There are 7 categories of MDS in the WHO system:
- Refractory cytopenia with unilineage dysplasia (RCUD)
- Refractory anemia with ringed sideroblasts (RARS)
- Refractory cytopenia with multilineage dysplasia (RCMD)
- Refractory anemia with excess blasts-1 (RAEB-1)
- Refractory anemia with excess blasts-2 (RAEB-2)
- Myelodysplastic syndrome, unclassified (MDS-U)
- Myelodysplastic syndrome associated with isolated del(5q)
Most of these categories are determined by the appearance of the cells in the blood and the bone marrow. One category is defined by a certain chromosome change in the bone marrow cells. Because small differences in the way the cells look can change the diagnosis, doctors may sometimes disagree on the exact MDS category for a patient's disease.
When a patient is first diagnosed with MDS their symptoms aren’t always apparent or easily diagnosed. Since everyone experiences this type of illness differently, some patients don’t even show signs or obvious symptoms of being ill right away. However, for patients who do show symptoms, these can usually relate to an abnormal blood count, which means that they have a low amount of red blood cells, white blood cells or platelets in their body.
For a person’s body to be in healthy state and free of illness like MDS, they must have the following:
- A proper amount of healthy white blood cells, which are essential to fight infections
- Platelets, which are needed in order to prevent ongoing bleeding
- Weakness
- Fatigue
- Dizziness
- Easy bruising
- Ongoing infections
- Receiving a Diagnosis
Causes of Myelodysplastic Syndrome
Some cases of myelodysplastic syndrome (MDS) are linked to known risk factors, but for most, the cause is unknown
Some genes (parts of DNA) contain instructions for controlling a cell's growth and division process. Certain genes that promote cell division are called oncogenes. Other genes called tumor suppressor genes can slow down cell division or even cause cells to die at an appropriate time. Cancers can be caused by DNA mutations (gene defects) that turn on oncogenes or turn off tumor suppressor genes.
Previous Cancer Treatment - Research has proven that patients, who have received prior cancer treatment, whether through radiation or chemotherapy, are at higher risk of developing MDS. People who endure stem cell transplants are also more at risk
Exposure to Benzene - Exposure to large amounts of harmful chemicals, such as benzene, can cause myelodysplastic syndromes. Benzene is an industrial chemical that’s found in gasoline, detergents, cigarette smoke and can even contaminate well water.
Treatment of Myelodysplastic Syndromes
The only known cure for MDS is allogeneic blood and marrow transplantation (BMT). Due to the older age of MDS patients and the intensity of allogeneic BMT, however, this treatment often isn't appropriate for MDS patients.
Allogeneic BMT achieves a cure through a combination of high-dose chemotherapy just before the transplant and an attack of the donor's immune cells against the patient's MDS cells, known as graft-versus-leukemia effect.
The major treatment strategies are:
- Supportive care
- Epigenetic therapy
- Biologic therapy
Supportive care entails transfusions of red blood cells and/or platelets, antibiotics for the prevention or treatment of infections, chelating agents to reduce the overload of iron that accompanies red blood cell transfusions, and growth factors.
There are two agents to unload iron: desferroxamine, which is given subcutaneously up to 12 hours daily, and deseferox (Exjade), which is given orally.
The red blood cell growth factors epogen (Procrit) and darbepoetic (Aranesp) do not work well to increase red blood cell production in MDS.
The white cell growth factors filgrastim (Neupogen), pegfligrastim (Neulasta) and sargramostim (Leukine) work well to increase white cell numbers and function, helping fight or prevent infection.
Epigenetic Therapy
Epigenetic therapy consists of the intravenous or subcutaneous administration of 5-azacitidine (Vidaza) or decitabine (Dacogen). These agents turn on tumor suppression genes that are silenced in MDS.
Tumor suppressor genes make proteins that normally prevent the emergence of cancer. In MDS, tumor suppressor genes are often silenced by the addition of chemicals called "methyl groups" to the DNA backbone (an epigenetic modification). Decitabine and 5-azacitidine prevent the addition of these methyl groups and reawaken the tumor expression genes, forcing MDS cells into the background.
Epigenetic therapy is slow — requiring months for a response — and requires indefinite use to remain effective. It does not cure MDS.
Biologic Therapy
Biologic therapy is currently limited to lenalidomide (Revlimid). Through unknown mechanisms, lenalidomide improves red blood cell production in certain patients with MDS, but at the expense of lowering white blood cells and platelets.
In MDS patients missing the long arm of chromosome 5 — the "5q minus syndrome" — lenalidomide eliminates the need for red blood cell transfusions two-thirds of the time. If the main goal is to improve red blood cell production, lenalidomide is likely to be a good treatment choice.
Stem Cell Transplantation
The only know cure for MDS is an allogeneic stem cell transplant, also called a bone marrow transplant. At UCSF, there are five different allogeneic transplantation protocols available for patients with MDS. Some are only available to younger patients (those under 55), but others are available to patients up to age 70 or even 75. These experimental protocols are aimed at evaluating the safety and effectiveness of allogeneic transplantation in older patients with MDS.
With allogeneic transplantation, it is expected that 20 percent to 30 percent of patients will die early of treatment-related complications, but that a minority of patients will be cured. Results seem to be better when the transplant is performed at an earlier stage of MDS and before the disease progresses to acute myeloid leukemia. Transplantation is being investigated in patients who have a related donor as well as with donors found through the National Marrow Donor Program (NMDP).
The advantage of autologous transplantation is that it does not require a donor. The disadvantage is that abnormal stem cells may be re-infused and cause MDS to develop again after the transplantation procedure.
Investigational Therapies
UCSF is dedicated to using investigational therapies and clinical research trials to improve outcomes for patients with MDS. UCSF participates in clinical trials combining epigenetic therapies with growth factors or biologic agents.
Lifestyle changes after Myelodysplastic Syndrome
You can't change the fact that you have had cancer. What you can change is how you live the rest of your life -- making choices to help you stay healthy and feel as well as you can. This can be a time to look at your life in new ways. Maybe you are thinking about how to improve your health over the long term. Some people even start during cancer treatment.
Making healthier choices
For many people, a diagnosis of cancer helps them focus on their health in ways they may not have thought much about in the past. Are there things you could do that might make you healthier? Maybe you could try to eat better or get more exercise. Maybe you could cut down on the alcohol, or give up tobacco. Even things like keeping your stress level under control may help. Now is a good time to think about making changes that can have positive effects for the rest of your life. You will feel better and you will also be healthier.
Eating better
If treatment caused weight changes or eating or taste problems, do the best you can and keep in mind that these problems usually get better over time. You may find it helps to eat small portions every 2 to 3 hours until you feel better. You may also want to ask your cancer team about seeing a dietitian, an expert in nutrition who can give you ideas on how to deal with these treatment side effects.
One of the best things you can do after cancer treatment is put healthy eating habits into place. You may be surprised at the long-term benefits of some simple changes, like increasing the variety of healthy foods you eat. Getting to and staying at a healthy weight, eating a healthy diet, and limiting your alcohol intake may lower your risk for a number of types of cancer, as well as having many other health benefits.
Rest, fatigue, and exercise
If you were sick and not very active during treatment, it is normal for your fitness, endurance, and muscle strength to decline. Any plan for physical activity should fit your own situation. An older person who has never exercised will not be able to take on the same amount of exercise as a 20-year-old who plays tennis twice a week. If you haven't exercised in a few years, you will have to start slowly – maybe just by taking short walks.
If you are very tired, you will need to balance activity with rest. It is OK to rest when you need to. Sometimes it's really hard for people to allow themselves to rest when they are used to working all day or taking care of a household, but this is not the time to push yourself too hard. Listen to your body and rest when you need to. (For more information on dealing with fatigue, please see Fatigue in People With Cancer and Anemia in People With Cancer.)
Keep in mind exercise can improve your physical and emotional health.
- It improves your cardiovascular (heart and circulation) fitness.
- Along with a good diet, it will help you get to and stay at a healthy weight.
- It makes your muscles stronger.
- It reduces fatigue and helps you have more energy.
- It can help lower anxiety and depression.
- It can make you feel happier.
- It helps you feel better about yourself.