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About MDS (Myelodysplastic Syndrome)

2/12/2018

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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.
  • Older age. Patients older than 60 have a higher risk of having the disease compared to younger patients.
  • Previous cancer treatment. Chemotherapy/radiation therapy, which are generally used to treat cancer, can lead to a higher rates of myelodysplastic syndromes.
  • Exposure to chemicals. Some substances have been associated with myelodysplastic syndromes. These substances include cigarette smoke, chemicals used to kill insects and manufacturing compounds, such as benzene.
  • Exposure to heavy metals. Those with a history of contact with heavy metals like lead and mercury have a higher risk of having Myelodysplastic Syndromes.
Symptoms
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.
  • Blood tests. A doctor might collect a small amount of blood. They will look at how many red/white cells and platelets a patient has. They will also look for irregularities in blood cells to try and detect disease.   
  • Bone marrow biopsy. A small amount of bone marrow is taken from the bone, typically from the hip bone, with a needle. The sample taken from the bone is called a biopsy. In a laboratory, the biopsy is examined for malformations in the cells.
Treatment Options
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.  
  • Blood transfusions. This procedure can be used to replenish red/white blood cells or platelets.
  • Growth factors. These drugs can help build up more blood cells in the body. They are artificial components that mimic of the body’s natural bone marrow factors.
Examples of growth factors that add to the number of red blood cells are epoetin alfa (Epogen, Procrit) or darbepoetin alfa (Aranesp). They can help reduce the number of transfusions a patient may need.  Examples of growth factors that add to the number of white blood cells are filgrastim (Neupogen, Zarxio). White blood cells help the body fight off infections.
  • Medications that stimulate blood cells. These medications help promote the maturation of blood cells. These drugs can lead to a better quality of life for patients with certain myelodysplastic syndromes and they lower the chances of getting certain types of leukemia (acute myelogenous leukemia). Examples of these medications include azacitidine (Vidaza) and decitabine (Dacogen)
  • Drugs that stifle the immune system. Certain medications can muffle the immune system. These medications can potentially reduce the number of blood transfusions that are needed for certain myelodysplastic syndromes.
  • Drugs that focus on a particular genetic abnormality. There are drugs that target a specific gene aberration called isolated del(5q). A drug that targets this specific mutation is called lenalidomide (Revlimid).
  • Bone marrow/stem cell transplant. Chemotherapy drugs destroy the aberrant blood cells in the bone marrow. The aberrant cells are then swapped with healthy transplanted cells from a donor.
  •  
References:
“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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About MDS (Myelodysplastic Syndrome)

5/1/2017

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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
  • pallor
  • weakeness
  • fatigue
  • fever and infections (neutropenia)
  • increased bruising
  • petechiae
  • bloody nose (epistaxis)
  • nosebleeds and bleeding gums caused by low platelet counts (thrombocytopenia)
  • splenomegaly (enlargement of the spleen)
  • hepatomegaly (enlargement of the liver)

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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