Myelodysplastic Syndrome

Posted by Graham McMahon • September 10th, 2010

This week’s Case Record, A 73-Year-Old Woman with Chronic Anemia, is presented by Drs. Eyal C. Attar and Robert P. Hasserjian of the Massachusetts General Hospital.

The myelodysplastic syndromes are diseases of the elderly, with diagnosis at a median age of 70 years. AML develops in approximately one third of persons with a primary myelodysplastic syndrome.

Clinical Pearls

What are the treatment goals for patients with myelodysplastic syndrome?

The main objectives of therapy are to control symptoms due to cytopenias, improve quality of life, and improve the overall survival while preventing or delaying progression to AML. Blood-product transfusions, colony-stimulating factors, and antibiotics (when required) provide the backbone of supportive care for patients with a myelodysplastic syndrome, either alone or during other treatments.

What treatments are available to manage patients with the myelodysplastic syndrome?

Three drugs have been approved by the Food and Drug Administration for the treatment of myelodysplastic syndromes. Two — azacitidine and decitabine — are hypomethylating agents. The third, lenalidomide, is an immunomodulatory agent derived from thalidomide. Other options for treatment include allogeneic stem-cell transplantation, which currently offers the only possibility for cure for patients with myelodysplastic syndrome.

Morning Report Questions

Q: What is the most common cytopenia in patients with the myelodysplastic syndrome?

A: Cytopenias in a myelodysplastic syndrome can evolve over a period of months or longer and may involve only single blood lineages; anemia is the most common cytopenia in myelodysplastic syndromes, followed by leukopenia and then thrombocytopenia.

Q: What is the difference in prognosis between primary and secondary myelodysplastic syndromes?

A: Myelodysplastic syndromes that arise with no preceding hematologic abnormalities or known exposures to chemotherapy or radiation, as in this patient, are called primary myelodysplastic syndromes. Alternatively, myelodysplastic syndromes may be secondary to toxic exposures, chemotherapy, or radiation used to treat other diseases such as malignant tumors and autoimmune disorders. Patients with secondary or therapy-related myelodysplastic syndromes have a worse prognosis than do patients with primary myelodysplastic syndromes.

Table 2. WHO Classification of Myelodysplastic Syndromes (MDS).

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