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What are Myelodysplastic Syndromes (MDS)?

Myelodysplastic Syndromes (MDS) are a group of diverse bone marrow disorders in which the bone marrow does not produce enough healthy blood cells.  MDS is often referred to as a “bone marrow failure disorder”.  MDS is primarily a disease of the elderly, but MDS can affect younger patients as well.

In the early stages, patients can have low blood counts or may need blood transfusions. But at more advanced stages, patients have a condition which is not dissimilar to Acute Myeloid Leukaemia.

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Causes of Myelodysplastic Syndromes (MDS)

In most cases, the exact cause of Myelodysplastic Syndromes may not be known. This is known as Primary MDS. When patients develop MDS after radiotherapy or chemotherapy, it is known as Secondary MDS or Treatment-related MDS.

Some factors are known to increase the risk of developing MDS:

  • Age: The incidence of Myelodysplastic Syndromes is higher in people above 60 years of age.
  • Exposure to toxic chemicals: Exposure to harmful chemicals can cause damage to the bone marrow and trigger abnormal cancerous changes that can lead to MDS. Some of these chemicals include:
    • Ionizing radiation, benzene and other industrial solvents.
    • Toxic chemicals present in cigarette smoke and pesticides.
    • Heavy metals like mercury and lead.
  • Exposure to Cancer Treatment Drugs: Patients receiving radiation therapy or chemotherapy for cancer treatment are more likely to subsequently develop Myelodysplastic Syndromes. MDS has been associated in particular in patients receiving prior treatment with a class of chemotherapy known as alkylating agents.
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Signs & Symptoms of Myelodysplastic Syndromes (MDS)

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Myelodysplastic Syndromes may not cause any signs and symptoms in the initial stages. As the disease progress, patients may experience symptoms related to impaired function of the blood such as:

  • Difficulty in breathing.
  • Persistent weakness and tiredness.
  • Tendency for bruising or bleeding due to thrombocytopenia or a low blood platelet count. This may also present as small red spots just beneath the skin called petechiae.
  • Unusual paleness of the skin due to anaemia or a low red blood cell count.
  • Frequent infections due to a low white blood cell count.

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Treatments for Myelodysplastic Syndromes (MDS)

The treatment for Myelodysplastic Syndromes depends on the stage of the disease, your age, and your performance status. It may involve one or a combination of therapies..

  • Growth Factor Injections
    Growth Factor Injections work by causing stimulation of the bone marrow. These drugs encourage the bone marrow to form an increased number of healthy white and red blood cells in the blood.
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    Growth factor injections containing erythropoietin may be recommended for patients who have a low red cell count. Erythropoietin can help to increase the production of red cells in the marrow and decrease the number of blood transfusions you might need.

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    A Growth Factor called G-CSF is usually administered to patients who have a low white cell count to stimulate the production of these cells.
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  • Blood Transfusions
    Blood transfusions include the transfusion of red cells or platelets aimed at improving the counts in the blood and relieve symptoms.
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    Transfusion of red cells is recommended for patients who experience severe symptoms of anaemia, such as unusual fatigue and shortness of breath. Patients who have received multiple blood transfusions are at risk of an accumulation of iron in the body resulting in an increased risk of longer term organ damage. Iron chelation may be required in such cases to remove the excess iron and reduce the iron overload.
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    Transfusions of platelets are recommended for reducing the risk of excessive bleeding due to a low platelet count. However, since platelets survive only for a few days, patients may need platelet transfusions more frequently.
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  • Immune Modulating Agents
    Administration of Immune Modulating Agents such as Lenalidomide, anti-thymoglobulin, and steroids are sometimes used in the treatment of low-risk MDS.
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    For example, Lenalidomide can be effective in reducing or eliminating the need for blood transfusions in some patients with Myelodysplastic Syndromes. Lenalidomide possesses anti-angiogenesis properties which means it can inhibit the formation of new blood vessels in the affected tissues, thereby preventing the survival of abnormal cells in the marrow. This would reduce the production of immature blood cells.
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  • Epigenetics Agents
    Epigenetic agents work by inhibiting cellular processes controlled by specific genes involved in the development of MDS. Epigenetic agents may reverse the genes that were suppressed in the bone marrow and help to restore the healthy functions of the bone marrow.
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    One of the epigenetic agents – 5-azacitidine, remains the only drug to have shown to improve overall survival in patients with MDS. 5-azacitidine is usually used in MDS patients with advanced disease, or MDS patients with low blood counts who may need blood product transfusions.
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  • Stem Cell Transplant
    Stem cell transplant involves the infusion of stem cells from the patient’s bone marrow or a donor. Stem cell transplantation is usually recommended for patients who have high-risk MDS. It is aimed at improving the long-term survival of patients.
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    A “matched related donor”, preferably a biological brother or sister of the patient having a similar genetic makeup, is considered the best source of stem cells for this procedure.

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FAQs on MDS

Blood tests

Blood tests can help to detect abnormalities in the blood cells counts. A reduced blood count can raise the suspicion of blood or marrow disorders.

Blood tests can also help to detect any abnormalities in the size, appearance, and shape of the blood cells.

Bone marrow biopsy

A bone marrow biopsy and aspiration involve the insertion of a thin needle into a long bone of the body to withdraw a small amount of marrow. The sample can be tested in a laboratory to detect any abnormalities linked to MDS.Specific tests are performed on the bone marrow aspirate including:

  • Immunophenotyping
    Immunophenotyping is a specialist test that is performed on either a blood sample or bone marrow aspirate sample. The patient sample is analysed through a machine known as a flow cytometer, which allows identification of abnormal markers which are present on the surface of white cells.

    Immunophenotyping allows for the detection of blood cancers, and aids in the classification of the sub-type of the blood cancers. In addition, as the test can detect even small amounts of residual disease in the blood or bone marrow, it is used following treatment to assess disease response.
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  • Cytogenetic tests
    Genetic tests of the bone marrow can help to confirm the diagnosis of MDS. The results of these studies can also be useful for determining how the disease will progress in the future.

    This test involves the examination of the chromosomes in the cells of the marrow sample taken during a biopsy. It can help to detect mutations in the chromosomes that can affect the functions of the bone marrow and trigger the development of MDS.
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  • Molecular testing
    Increasingly, there are several recognised molecular genetic abnormalities (mutations) in MDS cells that are linked to prognosis. Molecular testing provides information on the risk group of MDS patients. In addition, monitoring of the level of known mutations in patients often also allows us to measure the response of patients to therapy, even when there may be only very small levels of MDS disease in the bone marrow.

The prognosis of MDS is determined by several factors including the risk category, the presence of other medical conditions as well as age.

One of the most established prognostic scoring systems is the IPSS-R (International Prognostic Scoring System – Revised). This score is based on various patient factors including the haemoglobin level, neutrophil count, platelet count, bone marrow blasts and the cytogenetic category. The score helps to determine if a patient has low risk MDS or high risk MDS. Patients with low risk MDS tend to live for longer without the need for intervention. However, patients with high risk MDS generally require treatment sooner and may have a shorter life expectancy.

The IPSS-R (International Prognostic Scoring System – Revised) is a scoring system that helps to classify MDS patients at diagnosis into 1 of 5 groups. Patients are categorised from very low risk to very high risk groups based on 1) their risk of transformation to acute myeloid leukaemia (AML) and 2) risk of mortality.

Early-stage or low-risk MDS patients often have little or no symptoms from their MDS. Their blood counts may be lower than normal and they have a lower need for blood transfusion support.

Low-risk MDS patients usually have favourable or standard risk features on their bone marrow assessment for myeloblasts, cytogenetics and molecular mutations.

The IPSS-R (International Prognostic Scoring System – Revised) is a scoring system that helps to classify MDS patients at diagnosis into 1 of 5 groups. Patients are categorised from very low risk to very high risk groups based on 1) their risk of transformation to acute myeloid leukaemia (AML) and 2) risk of mortality.

Patients with high-risk MDS often have low blood counts and are symptomatic (lethargy, increased infections or bruising and bleeding).

High-risk patients will have an increased number of abnormal (myeloblast) cells in their bone marrow and often have poor risk features (such as high-risk cytogenetics or molecular mutations).

In the vast majority of cases, MDS is not inherited, meaning MDS is not passed from parent to child within a family.

The rate of progression of MDS to leukaemia is related to the severity of the underlying MDS. This is determined by factors including the extent of abnormalities in the full blood count, the genetic (cytogenetic) profile of the MDS, the mutation profile of the MDS (on next-generation sequencing), and whether there are blasts present on the bone marrow assessment.

Prognostic scoring systems like the IPSS-R (International Prognostic Scoring System – Revised) help to determine a patient’s risk of progression to AML.

Many patients with early or low-risk MDS may not have any symptoms, and the condition is sometimes found by blood tests done for other reasons.

However, many MDS patients may experience generalised symptoms, such as feeling tired or weak, losing weight without trying to, having a fever, getting black and blue marks or bleeding easily.

For patients with intermediate to high risk MDS, the disease will gradually progress without treatment, eventually leading to acute leukaemia, a life-threatening condition.

Some patients with very low risk MDS, on the other hand, may not require treatment or may only require supportive care with blood transfusions.

The prognosis of MDS is determined by several factors, including the risk category, the presence of other medical conditions, and age.

The IPSS-R (International Prognostic Scoring System – Revised) helps determine if a patient has low-risk or high-risk MDS. Patients with low risk MDS tend to live for longer without the need for intervention. However, patients with high risk MDS generally require treatment sooner and may have a shorter life expectancy.

The IPSS-R gives a general estimate of the time/risk of a patient to 1) develop acute myeloid leukaemia and 2) overall survival.

Many forms of lower-risk MDS can be managed with supportive care and/or treatments, with patients maintaining a good quality of life.

For patients with higher-risk MDS, though, the condition has an increased risk of progression to acute leukaemia over time. As such, bone marrow transplantation is the only curative option.

There are drug therapies for patients who are not eligible for transplantation to treat the underlying high-risk MDS effectively. However, there remains a high risk of disease relapse and progression.

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FAQs on MDS Treatments

At CFCH, we offer the following treatments for MDS: 

  • Blood transfusion
  • Growth factor injections
  • Immune modulating agents
  • Epigenetics agents
  • Stem cell transplant

The only curative form of treatment for MDS is an allogeneic haematopoietic stem cell transplantation (bone marrow transplant). However, not all MDS patients may be eligible for a transplant due to their age or other medical issues. 

For patients who are not suitable for a bone marrow transplantation, there are some medications which can help control the progression of the disease. If you have no symptoms, treatment might not be required immediately.

Low-risk MDS patients may not require any blood transfusions, and some subtypes of MDS patients with anaemia may respond well to medications that stimulate the production of red cells. There are also targeted drugs that improve the underlying MDS and boost the red cell count.

If you require transfusions, the frequency will vary between patients; some need transfusions every few months while others need one every couple of weeks. Very often, once a patient has started having regular blood transfusions, the length of time between transfusions will gradually get shorter.

These drugs encourage the growth of more healthy white and red blood cells. This will decrease the number of blood transfusions you may need.

Lenalidomide is used to treat low-risk MDS. It can inhibit the formation of new blood vessels in the affected tissues and prevent the survival of abnormal cells so that less blood transfusions are needed.

Epigenetics agents like 5-azacitidine inhibits the cellular processes controlled by genes that are involved in MDS development. By reversing the genes that are suppressed in the bone marrow, the epigenetics agents help to restore a healthy bone marrow.

Engraftment refers to transplanted stem cells settling into the bone marrow to begin creating new blood cells.

It typically takes between 10-14 days after the transplant for engraftment to occur.

A bone marrow transplant can fail if the body does not accept the new donor cells. This is known as graft failure and is suspected in patients whose counts do not increase after three to four weeks following a bone marrow transplant.

Graft failure can result in severe bleeding and infection and is more likely to occur in patients who receive a lower number of stem cells or when the patient and donor are not well matched.

Should a patient’s graft fail, they can receive a second dose of stem cells from the original donor. Sometimes a second transplant from a different donor is required.

The cost of a bone marrow transplantation depends on several factors, which include the type of transplant (autologous vs allogeneic), type of donor (related, vs unrelated, vs haploidentical) as well as the disease status of the patient and whether any potential complexities are expected for the procedure.

At CFCH, our team will work to establish the most appropriate treatment strategies for your condition. If a bone marrow transplantation is required, a detailed transparent breakdown of the costs will be provided upfront to you.

If you are concerned about the costs required for your bone marrow transplant, feel free to get in touch with us. We will be happy to help you understand the various fees involved.

MDS is a disease of the elderly, and the incidence of MDS increases significantly above the age of 60.

The only curative therapy for MDS remains an allogeneic haematopoietic stem cell transplantation (bone marrow transplant). However, many older patients are often unsuitable candidates due to the high risk of the disease.

The treatment for MDS in older patients depends on the stage of the disease, your age, and your overall physical status.

  • Supportive care, including blood transfusions and drugs to boost the blood counts
  • Immunosuppressive or immunomodulating agents (usually for lower-risk MDS)
  • Targeted chemotherapy drugs (usually for higher-risk MDS)
  • Stem Cell Transplantation

Our haematologists at CFCH will discuss the MDS treatment options with you and your family to work out the best-tailored treatment plan for you.

The cost of treating Myelodysplastic Syndromes varies depending on the therapy required. This is dependent on the stage of MDS, the age of the patient and the extent of co-morbidities of the patient.

Some MDS patients may not need any treatment or supportive care, while others may require intensive chemotherapy and even an allogeneic haematopoietic stem cell transplantation.

Our doctors at CFCH will evaluate your medical condition and assist in providing a detailed medical treatment plan, including the estimates of treatment costs.

At the present moment, an allogeneic haematopoietic stem cell transplantation is the only form of curative therapy for MDS.

However, other forms of less intensive therapies are available for MDS, which can often be effective in stabilising the condition and improving quality of life. There are newer directed therapies for MDS, such as the oral drug venetoclax, which has been used together with 5-azacitidine in the treatment of higher-risk MDS.

Our doctors at CFCH will evaluate your medical condition and assist in providing a detailed medical treatment plan, including the most appropriate therapies.

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Disclaimer:
The information on the Centre For Clinical Haematology website is intended for educational use.  It should not be considered or used as a substitute for medical advice, diagnosis or treatment from a qualified health professional.

Understanding Myelodysplastic Syndromes (MDS)

Video Credit: You and MDS

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Understanding Myelodysplastic Syndromes (MDS)

Video Credit: You and MDS

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    Locations

    Contact

    WhatsApp : +65 6256 8836
    Email : contact@cfch.com.sg
    .

    Consultation Hours

    Monday to Friday : 8.30am – 5.30pm
    Saturday : 8.30am – 12.30pm
    Closed on Sunday & Public Holidays

    Find us on Facebook

    Drop a Line

    If you have any questions about your condition or would like to make an appointment, simply fill up the form and we'll contact you as soon as we can

      © Centre for Clinical Haematology | 2023