Myelodysplastic Syndrome (MDS)
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What is MDS?
Myelodysplastic syndrome (MDS) is a disorder that is caused by bone marrow dysfunction. It can occur de nuvo (newly) or secondary (as a result of a treatment for another disease, such as cancer).
Indications of MDS are a low red and white blood cell count and low a platelet count where bleeding problems are present. Cancer is often the precursor.
Blood cells, red, white and platelets, are produced in the bone marrow. Immature blood cells are called "blasts" that normally develop into mature cells; however in MDS patients, the blasts do not develop properly. This causes an underproduction of red or white of blood cells, low platelet count, bleeding problems and/or a tendency to infection.
Some of the symptoms of MDS can include: chronic fatigue, shortness of breath, paler than usual skin, bruising and petechiae (flat, pinpoint spots under the sruface of the skin that are caused by bleeding).
Indications of MDS are a low red and white blood cell count and low a platelet count where bleeding problems are present. Cancer is often the precursor.
Blood cells, red, white and platelets, are produced in the bone marrow. Immature blood cells are called "blasts" that normally develop into mature cells; however in MDS patients, the blasts do not develop properly. This causes an underproduction of red or white of blood cells, low platelet count, bleeding problems and/or a tendency to infection.
Some of the symptoms of MDS can include: chronic fatigue, shortness of breath, paler than usual skin, bruising and petechiae (flat, pinpoint spots under the sruface of the skin that are caused by bleeding).
Contents at a Glance
Statistics
Between 10,000 and 15,000 people are diagnosed with MDS annually. 80% are over age 60.
What Blood Cells Do
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Red Blood Cells - carry oxygen throughout the body.
White Blood Cells - fight infection.
Platelets - control bleeding
Types of MDS
There are 7 types of MDS and life expectancy of the patient varies among the different types, from 6 months to several years. MDS transforms into acute myelogenous leukemia in 40 percent of patients.
The two most common types of MDS are refractory cytopenia and refractory anemia with ringed sideroblasts (RARS).
The two most common types of MDS are refractory cytopenia and refractory anemia with ringed sideroblasts (RARS).
- 1Refractory cytopenia with unilineage dysplasia (RCUD) - affects about 5% to 10% of MDS patients. Characterized by low red cell count but normal white count and platelet count. Prognosis is good; patients rarely if ever develop acute myeloid leukemia.
- 2Refractory anemia with ringed sideroblasts (RARS) - about 10% to 15% of MDS patients have this type. This type is similar to refractory anemia, however about 15% or more of the early red blood cells in the bone marrow contain ringed sideroblasts (cells that appear to have rings around the nucleus that indicate iron deposits).
- 3Refractory cytopenia with multilineage dysplasia (RCMD) - about 40% of MDS patients have this type and about 10% of the cases develop into leukemia. In RCMD, at least two types of blood cells are low. There is about a 50% survival rate within two years of diagnosis.
- 4Refractory anemia with excess blasts-1 (RAEB-1) - The number of blasts in the bone marrow for this type is 10% or less. There is about a 25% chance that RAEB-1 will develop into leukemia and the prognosis is poor; most patients die within two years.
- 5Refractory anemia with excess blasts-2 (RAEB-2) - This type is similar to RAEB-1 except that the number of blasts in the bone marrow is higher, about 10% to 20%. The chance that it could develop into acute leukemia can be as high as 50%.
- 6Myelodysplastic syndrome, unclassified (MDS-U) - This is a rare type and is basically a type wher the findings in the blood do not fit any other type of MDS. Since it's so rare, it hasn't been studied well.
- 7Myelodysplastic syndrome associated with isolated del(5q) - In this type of MDS, the red count is low, white count is normal and the platelet count is elevated. This type rarely develops into leukemia and the prognosis is very good.
"Refractory"
means that it is resistant to treatment.
Causes
MDS has been linked to radiation exposure, chemotherapy and gene mutation, radiation therapy, chemotherapy, tobacco smoke, pesticides and some solvents like benzene, but MDS can also occur for no apparent reason. Also, certain changes in the DNA of bone marrow have recently been found to cause MDS. Treatment
TransfusionsBlood and platelet transfusions are the primary components in a treatment plan for MDS along with the drug, Vidaza (azacitidine) or growth factors, such as filgrastim (G-CSF), sargramostim (GM-CSF), or erythropoietin (EPO) that stimulate the marrow to make more blood cells.
Transplant
A bone marrow transplant involves replacing defective cells. This can be a viable option for those for whom there is a compatible donor available and whose health is good enough to tolerate it.
Chemotherapy
Chemotheraphy is a harsh regimen and is usually perscribed for those with more severe cases and when other treatments are not possible. About half of those receiving chemotherapy go into remission, but the percentage of relapse is high and the long-term survival rate is low, especially in older patients.
Drug Therapies
The FDA has approved three drugs for the treatment of different types of MDS: azacitidine (brand name: Vidaza®), decitabine (brand name: Dacogen®) and lenalidomide (brand name: Revlimid®). (The names inside the parentheses are the trade names for these drugs.) Azacitidine and decitabine are approved to treat all types of MDS; lenalidomide is approved to treat only the 5q- syndrome type of MDS.
New Hope for MDS Patients
Help For Those with MDS
- MDS Foundation
- This organization was founded by physicians and researchers in order to facilitate the ongoing exchange of information about MDS
- National Cancer Institute
- Provides general information about MDS including treatment options.
- American Cancer Society
- Includes a detailed guide to MDS as well as news articles, clinical trials and stories of hope.
- Oncology STAT
- Includes links to medical journal articles on MDS, review journals and news.
For Further Information
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