Myelodysplastic Syndrome or MDS

Myelodysplastic Syndrome or MDS

  • People with Myelodysplastic Syndrome will have different symptoms depending on which blood cells are affected- a low red blood cell count is called anemia; a low white blood cell count is called leukopenia of these the  white blood cells that are the most important for attacking and killing bacteria  are called neutrophils,  a low neutrophil count is called neutropenia; a low platelet count is called thrombocytopenia.
  • Affects as many as 50 per 100,000 individuals over the age of 70. The disorder is five times more prevalent than AML, a form of leukemia.
  • More than 50 recurrently mutated genes are involved in the pathogenesis of MDS
  • MDS results from the clonal expansion of a hematopoietic progenitor and progresses to acute myeloid leukemia in approximately 30% of patients.

Blood is made of blood cells floating and plasma is the liquid part of the blood, and is mostly water but also contains proteins hormones minerals and vitamins. there are 3 types of blood cells in the blood, red blood cells are also called erythrocytes or red blood cells carry oxygen to cells and tissues; white blood cells, the body soldiers that fight infection; platelets are small cells that help the blood to clot.

Inside the bones is a spongy tissue, The bone marrow, which contains cells called stem cells, these cells are rapidly dividing forming 10 billion new blood cells every hour of every day.  These stem cells can grow copies or clones of themselves which eventually developed into mature blood cells once they are fully formed and functional the blood cells leave the bone marrow and go into the bloodstream.  In people with MYELODYSPLASTIC SYNDROME or MDS some of the stem cells in the bone marrow are abnormal most of the time we do not know the reason why stem cells become abnormal and this is called De Novo MDS.  We do note that certain factors are linked to MDS you are more likely to develop MDS; if you are a smoker, if you are heavily exposed to certain chemicals like pesticides or if you have received chemotherapy or radiation treatments for other cancers.

 

Abnormal stem cells may not develop intermittent your blood cells, instead, abnormal immature cells accumulate in the bone marrow where they crowd out the normal cells and decrease the body’s ability to form normal blood cells. Abnormal stem cells may also develop into abnormal or dysplastic blood cells- dysplastic means they have an abnormal shape size. These cells may not function properly and do not develop into mature blood cells efficiently. It is what is referred to in some circles as “Lazy Bone Marrow”.  The combination of bone marrow cells with immature cells as well as the presence of dysplastic cells that do not mature efficiently is the reason why patients with MDS have low blood counts.

Myelodysplastic Syndrome

Myelodysplastic syndrome (MDS) is a clonal disease that arises from the expansion of mutated hematopoietic stem cells. MDS is distinguished by the presence of peripheral blood cytopenias, dysplastic hematopoietic differentiation and the absence of features that define acute leukemia. More than 50 recurrently mutated genes are involved in the pathogenesis of MDS, including genes that encode proteins involved in pre-mRNA splicing, epigenetic regulation, and transcription.

People with MDS will have different symptoms depending on which blood cells are affected- a low red blood cell count is called anemia; a low white blood cell count is called leukopenia of these the  white blood cells that are the most important for attacking and killing bacteria  are called neutrophils,  a low neutrophil count is called neutropenia; a low platelet count is called thrombocytopenia

What are the risk factors for myelodysplastic syndrome?

Description of national incidence rates provides an important baseline for future studies of secular trends and allows for the examination of rates by selected demographic factors to define risk profiles of these hematologic malignancies in the American population.

 

What are the symptoms of myelodysplastic syndrome?

Myelodysplastic syndrome (MDS) often does not have symptoms in the early stages. If there are signs, they may be subtle or like those for other medical problems. When myelodysplastic syndrome does have symptoms, they may include:

  • Easy bruising
  • Fatigue
  • Weight loss, loss of appetite
  • Petechiae (tiny red spots just under the skin)
  • Fever
  • Frequent infections
  • Weakness
  • Shortness of breath

These symptoms do not always mean you have MDS.

 

 

How is myelodysplastic syndrome diagnosed?

The most common presentation is cytopenia. Diagnosis requires:

  • Peripheral blood examination 
  • Bone marrow aspirate and biopsy 
  • The diagnosis requires demonstration of dysplastic features in 1 or more cell line
  • Chromosomal abnormalities, Cytogenetic and gene mutations studies

once the Oncologies establishes a diagnosis and the given type of MDS, treatment can be initiated

The new classification is illustrated in the diagram below:

 

 

What are the treatments for myelodysplastic syndrome?

 

Patients with MDS are normally placed in two categories

Lower-risk myelodysplastic syndrome patients are treated initially for the specific complications of the disease, such as anemia and low blood counts. If more aggressive therapy is needed, strategies that are considered the standard of care include chemotherapy using hypomethylating agents such as 5-azacitidine/ decitabine and lenalidomide.

Higher-risk myelodysplastic syndrome patients usually need more aggressive therapy, but much depends on the age and condition of the patient. Younger patients with high-risk disease are considered for front-line chemotherapy approaches followed perhaps by allogeneic stem cell transplantation.

For older patients, intensive chemotherapy is rarely considered. Instead, our strategy focuses on the development of active and safe treatments for newly diagnosed patients as well as those for whom the standard of care based

 

The survival of MDS patients is poor, with an observed 3‐year survival rate of only 35. Male patients and patients who were diagnosed at an older age had significantly worse survival. MDS survival also varied by clinical subtype, and the survival of patients who had refractory anemia was somewhat worse than reported previously. The availability of descriptive epidemiologic data on MDS can be used now to facilitate much-needed research on the etiology and outcome of MDS. The current results indicated that >10,000 incident cases of MDS are diagnosed annually in the United States, and the survival of patients with MDS is poor.

 

The information in this document does not replace a medical consultation. It is for personal guidance use only. We recommend that patients ask their doctors about what tests or types of treatments are needed for their type and stage of the disease.

Sources:

  • American Cancer Society
  • The National Cancer Institute
  • National Comprehensive Cancer Network
  • American Academy of Gastroenterology
  • National Institute of Health
  • American Academy of Hematology
  • MD Anderson Cancer Center
  • Memorial Sloan Kettering Cancer Center

 

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