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Myelodysplastic syndromes are a cluster of diseases produced by dysfunctional or irregular blood cells. Myelodysplastic syndromes arise when there are irregularities in the spongy areas of the bones where blood cells are created, known as the bone marrow. Myelodysplastic syndromes are found in about 10,000 people per year in the US.
Based on the risk group (revised International Prognostic Scoring System (IPSS-R) risk groups) of a patient’s specific disease, the median survival period for patients can range from 8.8 years (very low risk) to less than a year (very high risk). A patient should discuss their risk group with their provider to get a median survival period specific to them. Median survival is a way to determine the potential results of a disease. It is the time after diagnosis in which 50% of patients are still alive and 50% have passed. It is a middle value. Fifty percent of the patients will live longer than the median survival period, and 50% of the patients will not live past the median survival period. Risk Factors There are factors that increase the chances of having Myelodysplastic Syndrome.
The symptoms associated with Myelodysplastic Syndromes include the following: feelings of tiredness, shortness of breath, uncharacteristic pastiness/paleness due to anemia (low blood cell count), uncommon bruising/bleeding due to low platelet counts in the blood, petechiae (tiny spots on the skin caused by bleeding under the skin), repeated infections due to leukopenia (a low number of white blood cells in the blood). Diagnosis If a doctor thinks a patient has myelodysplastic syndrome, s/he may conduct tests to confirm a diagnosis. The tests could include several things.
If a patient has no symptoms, with a doctor’s consultation, the patient may decide to take the watchful waiting approach. This means the patient will not be on treatment but they will get frequent evaluations and undergo diagnostic checks to monitor the progress of their disease.
“Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version.” National Institutes of Health National Cancer Institute, 2019. https://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq#_1 . “Survival Statistics for Myelodysplastic Syndromes.” American Cancer Society, 2019. https://www.cancer.org/cancer/myelodysplastic-syndrome/detection-diagnosis-staging/survival.html . “Myelodysplastic syndromes.” Mayo Foundation for Medical Education and Research, 2019. https://www.mayoclinic.org/diseases-conditions/myelodysplastic-syndrome/symptoms-causes/syc-20366977 Web links about Myelodysplastic Syndromes Patient education: Myelodysplastic syndromes (MDS) in adults (Beyond the Basics) https://www.uptodate.com/contents/myelodysplastic-syndromes-mds-in-adults-beyond-the-basics Myelodysplastic Syndromes Treatment (PDQ®)–Patient Version https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq Myelodysplastic Syndrome (MDS) https://www.merckmanuals.com/home/blood-disorders/leukemias/myelodysplastic-syndrome-mds Myelodysplastic syndrome https://medlineplus.gov/ency/article/007716.htm What Are Myelodysplastic Syndromes? https://www.cancer.org/cancer/myelodysplastic-syndrome/about/what-is-mds.html
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Myelodisplastic Syndrome involves a group of disorders that are often called preleukemia. It is not generally known what puts someone at higher risk for MDS. Exposure to benzene, radiation and chemotherapy drugs are sometimes associated with MDS.
The most common symptom is anemia, an abnormally low number of white blood cells (neutropenia), and thrombocytopenia or a combination. Patients with MDS often develop severe anemia and need frequent blood transfusions. In most cases, the disease worsens and the patient develops low blood counts (cytopenias) due to progressive bone marrow failure. In about one third of patients with MDS, the disease transforms into acute myelogenous leukemia (AML), usually within months to a few years. The myelodysplastic syndromes are all disorders of the stem cell in the bone marrow. In MDS, blood production (hematopoiesis) is disorderly and ineffective. The number and quality of blood-forming cells decline irreversibly, further impairing blood production Each year between 15,000 and 20,000 new cases of MDS are diagnosed in the US. Patients tend to be around the age of 70 at the time of diagnosis, although people of any age can develop the disease. MDS is classified as either primary (also called de novo) — disease that has no known cause — or secondary (also called treatment-related) MDS. Treatment-related MDS is caused by previous treatment with chemotherapy or radiation therapy, which is given for a previously diagnosed cancer. The number of new cases of MDS is rising, both because people are getting older and are now living longer after treatment for their first cancer. Symptoms
Diagnosis In order to diagnose MDS your doctor will ask you questions about your past health and symptoms. Your doctor will do a physical exam. MDS is suspected in elderly patients with refractory anemia, leukopenia or thrombocytopenia. Testing such as a complete blood count and bone marrow examination are completed. Treatment The treatment will try to eliminate or help the symptoms. Azacitidine is a medicine that relieves symptoms, decreases the rate of transformation to leukemia and the need for transfusions, and it probably also improves survival. About 16% of patients had a complete or partial response. Other therapy can include red blood cell (RBC) transfusions, platelet transfusions for bleeding, and antibiotics for bacterial infections. Another similar drug Deoxyazacitidine is sometimes effective. About 20-40% of patients had a partial response to the medicine. Some patients require erythropoietin, a medicine, to support their red blood cell production. When there is a severe decrease in white cells Granulocyte colony stimulating factor (GSF) can be used, which is a growth factor produced by a number of different tissues to stimulate the bone marrow to produce granulocytes and stem cells.Thrombopoietin, a medicine that regulates production of platelets by the bone marrow can be used for severe thrombocytopenia. Allogeneic stem cells transplants are useful. |
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