Myelodysplastic Syndrome
Myelodysplastic syndromes (MDS) are a group of several different conditions all caused by defective blood cells. Myelodysplastic syndromes are brought about when a problem develops in the bone marrow, the material inside the bones where blood cells are produced.
Because their symptoms are so similar, some forms of MDS can be confused with another benzene related illness, aplastic anemia. The primary difference between myelodysplastic syndromes and aplastic anemia is this: in cases of MDS, the bone marrow makes dysfunctional or immature blood cells that work improperly; in patients with aplastic anemia, the bone marrow simply stops making new blood cells at all. Another difference is that myelodysplastic syndromes are seen more often in older people; young people are more likely to be afflicted with aplastic anemia.
At present, no cure exists for myelodysplastic syndromes. Their treatment generally emphasizes the reduction or prevention of complications associated with the condition. In some cases, doctors may recommend a bone marrow transplant, which, when successful, can prolong the patient’s life.
Symptoms
The symptoms of myelodysplastic syndromes are not generally seen until the later stages of the disease. When they do appear, the symptoms frequently include:
• Fatigue;
• Frequent infections;
• Unusually pale skin;
• Bleeding or bruising that is caused easily or is frequently occurring;
• Red pinpoint spots just below the skin’s surface, caused by bleeding (known as petechiae); and
• Shortness of breath.
Should you develop any of these symptoms and become concerned about your health, make an appointment with your physician.
Causes
Myelodysplastic syndromes appear when the normal production of blood cells is disrupted. Myelodysplastic syndromes produce immature and defective blood cells; rather than developing normally, the blood cells die either in the bone marrow or shortly after entering the bloodstream. As the disease progresses, the problematic cells outnumber the healthy ones.
Myelodysplastic syndromes are categorized based on their cause. Myelodysplastic syndromes without a known cause are referred to as de novo myelodysplastic syndromes. These conditions are easier to treat than those with a known cause.
Diagnosis
When a patient is found to have unusual blood cell counts, it is likely that further tests and procedures will be performed to discover the cause of any abnormality. Generally, doctors will perform some, or all, of the following test and procedures:
Complete Blood Count. Blood tests may be used to count the number of blood cells found in a sample of the patient’s blood.
Peripheral Blood Smear. Blood samples may be examined to look for unusual changes in the shape, size and appearance of certain blood cells.
Bone Marrow Testing. A bone marrow biopsy and aspiration is performed by inserting a thin needle into the back of the hipbone to withdraw liquid bone marrow and a small sliver of bone. The biopsy materials are then examined in a laboratory to determine whether abnormalities exist.
Treatments
Because there is no known cure for myelodysplastic syndromes, most treatments are geared toward managing symptoms and complications of the disease. Your doctor may recommend some or all of the following:
Blood transfusions. Blood transfusions are used to replace dysfunctional cells with healthy red blood cells, white blood cells or platelets.
Medications. Several medications exist that have proven useful in boosting the number of healthy blood cells produced by the body, including:
• Growth factor medications – These artificial versions of substances naturally occurring in bone marrow increase the number of blood cells made in the body. Erythropoietin and darbepoietin, for example, may cut back on the need for blood transfusions by increasing the number of red blood cells produced. Other growth factors help stave off infections by increasing the number of white blood cells.
• Medications to stimulate cell maturity – Some medications, like decitabine (Dacogen) and azacitidine (Vidaza), help blood cells grow to maturity, when the cells might otherwise remain immature. These drugs may improve the patient’s quality of life and work to delay the onset of acute myelogenous leukemia (AML). Still, the medications are not always effective and sometimes cause additional blood cell difficulties.
• Medications to suppress the immune system.
• Medications to address a genetic abnormality – For patients whose myelodysplastic syndrome is linked to isolated del(5q), doctors sometimes recommend lenalidomide (Revlimid) to reduce the necessity for blood transfusions.
Bone marrow/stem cell transplant. A bone marrow, or stem cell, transplant begins with the destruction of the defective blood cells with powerful chemotherapy agents. The dysfunctional bone marrow cells are then replaced with the healthy blood cells of a compatible donor. When a donor’s cells are used, as opposed to the patient’s own cells, the process is called an allogeneic transplant. Due to the high risks associated with transplants in older patients, the bone marrow transplant success rate is not very encouraging. Most patients with myelodysplastic syndromes are older, and even among young people with otherwise good health, transplant-related complications are still quite frequent.
Healthy Lifestyle Options
Myelodysplastic syndrome patients who suffer from low white blood cell counts are likely to experience recurrent infections. To avoid the risk, it is best to develop certain simple habits for a healthy lifestyle. Wash your hands frequently, especially before eating or cooking a meal. Thoroughly wash and cook your food. Avoid close contact with people who are ill.
If you have been diagnosed with myelodysplastic syndrome and you suspect that it may be related to benzene exposure, please contact us for a free consultation. You should know your legal rights.