Myelodysplastic Syndrome

What is Myelodysplastic Syndrome?

This is a group of conditions caused by dysfunctional or poorly formed blood cells. These syndromes happen when an event of some sort goes wrong in the bone marrow which is the spongy matter inside the bones and is where blood cells are created.


There is no cure for this group of syndromes. Treatment for these syndromes normally will focus on preventing or reducing any complications of the disease and its management. In some cases, these syndromes are managed with a bone marrow transplant that might help in prolonging life.

Symptoms

These syndromes very rarely cause symptoms or signs in the early stages of the disease. After time, the syndromes might cause:

  • Shortness of breath
  • Fatigue
  • Unusual paleness or pallor due to anemia
  • Unusual or easy bleeding or bruising
  • Red spots that are pinpoint-sized right beneath the skin cause bleeding – petechiae
  • Infections that are frequent

Causes

These syndromes happen when something causes disruption of the controlled and orderly production of blood cells. Individuals with these syndromes have cells of blood that are defective and immature and rather than normally developing, they often die while in the bone marrow or right after entrance into the blood system. As time passes, the amount of defective, immature, blood cells start to exceed those blood cells that are healthy leading to situations such as infections, excess bleeding, and anemia.

The medical literature has divided these syndromes into 2 categories based on their causes:

Myelodysplastic Syndromes no cause known – this is called “de novo myelodysplastic syndrome”, and physicians do not know the causal factor. These syndromes often are easier to treat than are the syndromes where a cause is known.

Myelodysplastic Syndromes caused by radiation or chemical – these syndromes occur in reply to treatments such as chemotherapy or radiation or in reaction to exposure chemically called “secondary myelodysplastic syndromes”. These secondary syndromes are frequently harder to manage or treat.

Types of Myelodysplastic Syndromes

The WHO or World Health Organization separates these syndromes into sub-types based on the blood cells type – red cells, platelets and white cells – involved. These syndrome sub-types consist of:

Refractory cytopenia w/unilineage dysplasia (RCUD)

With this syndrome, one type of blood cell is lower in number than the others. This type of blood cell emerges atypically under the microscope.

Refractory anemia with ring sideroblasts (RARS)

This syndrome entails a low amount of red blood cells. The existing red blood cells hold disproportionate amounts of iron.

Refractory cytopenia w/ multilineage dyplasia (RCMD)

With this syndrome, 2 of the 3 blood cells types are atypically, and less than 1% of the cells of the blood system are immature cells or blasts. There is a propensity to become acute myeloid leukemia (AML).

Refractory anemia with excessive blasts (RAEB)

In each of these types, any of the 3 types of blood cells – white blood cells, red blood cells, or platelets – can be reduced in numbers and are visible atypical under a microscope. Very young blood cells or blasts are found in the blood.

Myelodysplastic syndrome, unclassified (MDS)

This is a very rare syndrome, with decreased numbers of 1 of the 3 types of older blood cells and the white cells or the platelets look atypical with the microscope.

Movement to AML leukemia is less common in MDS with isolated del (5q) than in any other forms of MDS.

Treatment

When numerous abnormal blood cells have been discovered in the blood, your physician may start with tests and measures to rule out circumstances and diseases other than these syndromes that have symptoms and signs similar.

Additionally, the physician may advocate definite tests and measures used to diagnose these syndromes, such as:

Blood Tests

The physician may advocate blood testing to calculate the number of blood cells in your blood sample (CBC or complete blood count) and look at the blood for strange alterations in the shape, size and look of various cells of blood (peripheral blood smear).

Removal of marrow of the bone to test

During a biopsy of bone marrow, a physician or nurse will use a needle that is thin in order to aspirate a tiny amount of bone marrow, normally starting at a spot on the back of the hipbone. A small bit of bone and its marrow also is taken away for testing (biopsy). The sections are looked at in a lab to look for problems.

No definitive treatment

There is no definitive treatment or cure for these syndromes. Instead, most individuals receive compassionate care so as to manage symptoms such as tiredness and to stop infections and bleeding.

Transfusions of blood

These may be used to restore red cells, white cells, or platelets in individuals with these syndromes.

Medications

Medications to increase the amount of cells in the blood that are healthy that the body creates include:

  • Medications to increase the amount of blood cells the body makes. Referred to as factors for growth, these drugs are artificial adaptations of materials found normally in the marrow of the bone. Some of these factors for growth, such darbeopoetin alfa (Aranesp) or as erythropoietin alfa (Epogen), may decrease the need for transfusions of blood by escalating red cells. Others may help stop infections by increasing white cells in individuals with certain syndromes.
  • Medications that motivate blood cells to reach their prime, instead of remaining immature. Drugs such as decitabine (Dacogen) and azacitidine (Vidaza) might advance the life quality for individuals with some of these syndromes and aids in delaying any progression to AML leukemia. But not all individuals are help with these medications and some can even cause additional blood problems.
  • Medications that repress the system of immunity. Drugs used to repress these immune system might be used in some syndromes.
  • Medication for individuals with a definite abnormality genetically. If your syndrome is linked to an abnormality that is genetic called “isolated del (5q)”, the physician might advocate lenalidomide (Revlimid). This drug can reduce any need for transfusions in individuals with this abnormality.

Marrow of the bone stem cell transplant – With this transplant, the blood cells that are defective are destroyed using chemotherapy drugs. Then the atypical blood marrow is restored with healthy, cells donated (allogeneic transplant). Regrettably few individuals are nominees for this process due to the high hazards involved in transplantation in older adults – those more likely to have these syndromes. Even amid young, relatively healthy individual, the risks of transplant-related problems are high.

Prognosis

The prognosis or chance of recovery is dependent on the following:

  • If the syndrome developed after radiation therapy or chemotherapy for another problem
  • The amount of blast cells in the marrow of the bone
  • Whether only one or more types of cells of blood are affected
  • Certain change in chromosomes

Life expectancy

Life expectancy at the time of diagnosis dependent on the types of cells that are affected.

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