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Myelodysplastic Syndromes
Myelodysplastic syndromes, a group of cancers of the blood and bone marrow, are expected to afflict about 11,000 individuals in the United States this year. The overall incidence rate is 4.3 per 100,000.
*Source: Leukemia & Lymphoma Society
 

Myelodysplastic Syndromes Overview

WellStar Health System provides superior care for myelodysplastic syndromes, offering a comprehensive spectrum of top-notch physicians, treatment options and diagnostic tools. In addition WellStar offers such innovative advances in the fight against myelodysplastic syndrome, including:


  • Advanced oncologists and hematologists specializing in blood cancers and who practice state-of-the-art care in a collegial atmosphere.
  • A Tumor Board, which weekly meetings of a multidisciplinary team, for review of complex cases
  • Clinical trials - making novel therapies available to patients.

Myelodysplastic syndromes (MDS) are a group of disorders caused by poorly formed blood cells. It originates in the bone marrow, where blood cells production has become disordered and ineffective.


Normally, bone marrow produces blood stem cells that eventually develop into mature blood cells. When a blood stem cell divides, it makes two cells – a stem cell, and either a lymphoid stem cell, which develops into a lymphocyte, a type of white blood cell; or a myeloid stem cell, which can develop into either:


  • A red blood cell, which carries oxygen and help carry carbon dioxide
  • A type of white blood cell that fight infections
  • A platelet, which enables stops bleeding

In myelodysplastic syndromes, the stem cells do not mature properly into healthy, useful blood cells; dysplasia refers to abnormal development. The immature cells, called blasts, do not function normally, and die in the marrow or shortly after they enter the blood stream. This leaves a shortage of normal blood cells, causing a range of health problems.


In about a third of cases, MDS develops into a fast-growing cancer of bone marrow cells called acute myeloid leukemia. In the past, MDS was called pre-leukemia or smoldering leukemia; however, since most MDS patients do not get leukemia, MDS is regarded to be a separate form of cancer.


MDS are classified by abnormalities in the marrow and blood cells:


  • Refractory (hard to treat) anemia (RA): Too few red blood cells in the blood causing anemia; quantity of white blood cells and platelets is normal.
  • Refractory anemia with ringed sideroblasts: Too few red blood cells in the blood causing anemia and these cells have too much iron; quantity of white blood cells and platelets is normal.
  • Refractory anemia with excess blasts: Too few red blood cells in the blood causing anemia. Five to 19 percent of bone marrow cells are blasts, but there is a normal quantity of blasts in the blood. There also may be abnormalities in the white blood cells and platelets. May progress to acute myeloid leukemia.
  • Refractory anemia with excess blasts in transformation: Too few red blood cells, white blood cells, and platelets in the blood. Anemia. Twenty to 30 percent of the cells in the bone marrow are blasts and more than five percent of the cells in the blood are blasts. Refractory anemia with excess blasts in transformation is also called acute myeloid leukemia.
  • Refractory cytopenia with multilineage dysplasia: Too few of at least two types of blood cells in the blood. Fewer than five percent of the cells in the bone marrow and less than one percent of cells in the blood are blasts. If red blood cells are affected, they may have extra iron. May progress to acute leukemia.
  • Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality: Too few red blood cells in the blood causing anemia; fewer than 5% of the cells in the bone marrow and blood are blasts. There is a defect in the del(5q) chromosome of the cells' DNA.
  • Unclassifiable myelodysplastic syndrome: There are too few of one type of blood cell in the blood, but the quantity of blasts in the bone marrow and blood is normal, and the disease is not one of the other MDS syndromes.

Myelodysplastic syndromes are also classified by apparent cause:


  • If there is no apparent cause, they are called de novo myelodysplastic syndromes. These are usually more easily treated.
  • If there is an apparent cause, such as cancer treatment or chemical exposure, they are called secondary myelodysplastic syndromes. and are usually more difficult to treat.

Symptoms

Symptoms of myelodysplastic syndromes mimic those of many other illnesses, making the disease hard to diagnose. Sometimes, there are no symptoms, and the disease is discovered through a routine complete blood count (CBC).


Symptoms of MDS include:


  • Fatigue.
  • Shortness of breath during physical activity.
  • Skin that is paler than usual.
  • Easy bruising or bleeding.
  • Pinpoint spots under the skin caused by bleeding, known as petechiae.
  • Fever or frequent infections.

Risk Factors

  • Radiation treatment or chemotherapy
  • Exposure to benzene or certain pesticides. Cigarette smoke is the leading known carrier of benzine; industrial exposure is strictly limited.
  • Exposure to metals such as mercury and lead
  • Being male
  • Being older than 60 (80 to 90 percent of MDS occur in people over 60)
  • Family history of MDS

Most people who are exposed to benzene, radiation treatment, or chemotherapy do not develop MDS; those who do may have inherited an inability to detoxify the causative agent.