Life Expectancy of Someone With Myelodysplastic Syndrome

People with low-risk myelodysplastic syndromes (MDS) can live for five years or more with current treatments. Those with high-risk MDS, who develop acute myeloid leukemia (AML), have a reduced life expectancy.

MDS has various subtypes detected by blood and bone marrow tests. Your therapy and prognosis depend on the MDS subtype.

The International Prognostic Scoring System-R is often used by doctors to determine prognosis. It categorizes MDS into five risk groups.

This score system helps determine the severity of the disease and the probability of progression to AML. The doctor can estimate your life expectancy based on this score.

This system considers three factors to determine the subtype of MDS:

1. The type of blood cells that have become abnormal
2. Red blood cells
3. White blood cells
4. Platelets
5. Percentage of immature blood cells (blasts) in the body
6. The presence of a chromosomal abnormality called deletion 5q in the blood cells

What is myelodysplastic syndrome?

Myelodysplastic syndromes (MDS), also called myelodysplasia, are malignancies that develop when the bone marrow fails to create enough normal blood cells to sustain the body.

MDS is one of the most common blood cell disorders in older adults. One in three people with MDS will develop acute myeloid leukemia. However, for some, MDS is a chronic illness that can be managed with medication.

How does myelodysplastic syndrome develop?

Bone marrow, a soft tissue in the bones, contains stem cells that produce healthy blood cells, including red blood cells, white blood cells, and platelets. With certain genetic abnormalities, the bone marrow loses the ability to produce healthy blood cells and instead produces immature cells called blasts. This condition leads to the development of myelodysplastic syndromes (MDS).

These blasts are short-lived or too deformed (dysplastic) to function. They die in the bone marrow or shortly after entering circulation.

The affected person experiences fatigue when the blood cell count is low, and vital blood functions such as oxygen delivery, fighting infections, and clotting are affected. MDS can manifest as anemia, low platelets, or a low white blood count in its mildest form, but 10 to 20 percent of identified MDS cases progress to acute myeloid leukemia (AML). It is considered a pre-leukemic disease.

Types of myelodysplastic syndrome

The World Health Organization classifies myelodysplastic syndrome (MDS) into subgroups depending on the blood cells involved.

Subtypes of MDS

• Myelodysplastic syndromes with single-lineage dysplasia

• Only one type of blood cell is low in number and shows abnormality under the microscope.
• Two or three types of blood cells are identified to be abnormal.
• The count of one or more types of blood cells is low.
• Red blood cells in the bone marrow are significantly high in iron, forming rings.
• People with this subtype have a low amount of red blood cells, and the cell’s DNA has a unique mutation.
• Any one type of the three types of blood cells exhibits a low count and abnormality.
• Blasts (immature blood cells) are found in bone marrow and blood.
• The count of one or more types of mature blood cells is low and is identified as abnormal under a microscope.
• Some blood cells may appear normal, but chromosomal analysis may reveal DNA mutations associated with MDS.

Types of pediatric MDS

• Refractory cytopenia of childhood: Fewer than five percent of blasts are present in the bone marrow.
• Refractory anemia with excess blasts (RAEB): 5 to 20 percent of blasts are present in the bone marrow.
• RAEB in transformation (RAEB-t): 21 to 30 percent of blasts are present in the bone marrow.

Causes and risk factors of myelodysplastic syndrome

Myelodysplastic syndrome (MDS) is classified as primary and secondary MDS. The cause of primary MDS is unknown. Secondary MDS develops following aggressive treatment to cure other malignancies in the body. This includes exposure to:

• Radiation
• Alkylating agents
• Topoisomerase II inhibitors
• Autologous bone marrow transplants

Risk factors that increase the odds of developing MDS

• Advancing age: Usually develops in adults older than 60 years.
• Gender: Men are more prone to developing MDS than women.
• Genetic predisposition:

• MDS can run in the family.
• Other diseases caused by inherited genetic mutations can increase the risk of MDS.
• Genetic counseling can detect the risk of acquiring the condition.
• Fanconi anemia
• Aplastic anemia
• Noonan syndrome
• Down syndrome
• Shwachman-Diamond syndrome
• Neurofibromatosis type 1
• Familial platelet disorder
• Severe congenital neutropenia
• Amegakaryocytic thrombocytopenia or familial thrombocytopenia
• Exposure to certain environmental factors can cause permanent injury to the hematopoietic stem cells in the bone marrow and lead to MDS. These include:

• Smoking tobacco
• Long-term chemical exposure, such as benzene and chemicals used in the petroleum and rubber industries
• High-level radiation exposure, such as a nuclear reactor accident or an atomic explosion
• People who have undergone both chemotherapy and radiation to treat another cancer are at a significantly high risk of developing MDS.
• This does not mean everyone who undergoes these treatments develops MDS.

MDS does not affect everyone with risk factors. However, it is recommended to discuss your risk factors with your doctor.

Symptoms of myelodysplastic syndrome

Early stages of myelodysplastic syndrome (MDS) may not display any signs or symptoms. Some people may experience mild symptoms that gradually worsen over time.

The symptoms that arise depend on the type of blood cell most impacted. Most people with MDS commonly have anemia (reduced red blood cell count). A low white blood cell count (neutropenia), a low platelet count (thrombocytopenia), or both can indicate MDS.

Many symptoms of MDS are similar to other medical conditions. Consult a doctor to determine the cause.

Most common symptoms of MDS

• Neutropenia (fever and recurrent infections due to a reduced number of white blood cells)
• Thrombocytopenia (low number of platelets)

• Increased risk of bleeding
• Easy bruising
• Petechiae (tiny red spots just under the skin)
• Shortness of breath, especially during exercises
• Fatigue
• Pale skin
• Weakness
• Headache
• Increase heartbeat

Some individuals are identified with MDS during routine blood workup for other causes and usually before experiencing any symptoms.

Some individuals are identified with MDS during routine blood workup for other causes and usually before experiencing any symptoms.