Dr. M.J. Bazos, Patient Handout
MYELODYSPLASTIC SYNDROME
About Your Diagnosis
Myelodysplastic syndrome is a proliferation of abnormal bone marrow cells that leads to acute leukemia. There are five subtypes of this condition. Type I and Type II are characterized by refractory anemia. The other three types have an excessive number of blasts (leukemia cells). The higher the percentage of blasts, the shorter is the interval to development of leukemia. The cause is unknown in most instances. Chemotherapy and radiation therapy can be causative for so-called secondary myelodysplastic syndrome. Myelodysplastic syndrome is uncommon. Among persons older than 60 years the incidence is 0.75 per 1000 per year. Less than 7% of patients are younger than 50 years. The disease occurs randomly. Some families have a predisposition to myelodysplasia. Patients with anemia and low blood counts need to undergo examination. A blood smear review and examination of the bone marrow are necessary for diagnosis. Increased numbers of early blood cell precursors (blasts) are predictors of advanced disease. Special genetic analysis of the bone marrow cells helps define who has a poor prognosis. Some young patients with a good prognosis can be cured with bone marrow transplantation. Combinations of chemotherapy and growth factors can cause remissions and improve the symptoms.
Living With Your Diagnosis
About 50% of patients have no symptoms. The most frequent sign is anemia (low red blood cell count and hemoglobin). Two thirds of patients also have a low white blood cell count or platelet count. With time 30% of patients have acute leukemia. Pallor, excessive fatigue, and shortness of breath with exertion can occur with severe anemia. A low platelet count can lead to bleeding. The main risk of a low white blood cell count is development of serious infections.
Treatment
Supportive care with regular transfusions and antibiotics for infections is the mainstay of therapy. Transfusions with red blood cell and platelet concentrates support patients with low blood cell counts. Chemotherapy regimens with cytarabine, azacitidine, and etoposide can produce remissions,
but the remissions are only temporary. Use of growth factors, erythropoietin, and filgastrim (Neupogen) can decrease the number of transfusions and episodes of infections for some patients. Different agents, such as vitamin A and D analogs, interferon, steroids, and androgen hormones can be tried, but they have limited success. Supplements with folic acid and vitamin B6 (pyridoxine) are beneficial. A small proportion of young patients benefit from bone marrow transplantation. Chemotherapeutic drugs, which can decrease blood counts even more during treatment, cause nausea and vomiting. Special antinausea medications and blood cell growth factors can prevent these effects. Bone marrow transplantation can have toxic effects on the liver, lungs, and brain and predispose to infections. The most serious complication of transplantation is graft-versus-host disease, in which the bone marrow cells of the donor attack the patient. This can be prevented and treated with immunosuppressive drugs. Long-term use of transfusions can lead to iron overload. A special iron-excreting drug can be administered with the transfusions.
The DOs
• Consider the option of bone marrow transplantation, either from a close relative or a suitable unrelated donor. The risks and benefits of this procedure are different for individual patients.
• Obtain a vaccination for hepatitis if you are undergoing transfusions. Revaccination is needed after transplantation.
• Use medical alert identification.
• Discuss contraceptive measures with your physician.
• Inform household members not to be vaccinated with live viruses (eg, polio) if you have undergone bone marrow transplantation.
The DON’Ts
• Avoid use of aspirin and aspirin-like medications; they can worsen the bleeding. Discuss use of other medications with your physician. Some medicines can lower blood counts.
• Avoid fresh vegetables and fruit, cheese and yogurt if you have a low white blood cell count.
• Avoid moderate and strenuous exercise if you have severe anemia.
• Avoid interactive and potentially traumatic activities if you have a low
platelet count.
• Avoid large crowds and persons who have signs of infections if you have a low white blood cell count.
When to Call Your Doctor
• If you experience fever, bleeding, chest pain, or dizziness.
Websites: http://www.cancer.org
http://medmark.bit.co.kr/hematolo.html
http://nysernet.org/bcic/bmt.news.html