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Idiopathic Aplastic Anemia
Aplastic Anemia

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Definition

Aplastic anemia is a condition in which there is failure of the bone marrow to properly produce all types of blood cells i.e. red blood cells, white blood cells and platelets. This type of anemia is also called pancytopenia.

Aplastic anemia results from injury to the stem cell in the bone marrow. Stem cells give rise to other cell types in the bone marrow after it divides and differentiates. Consequently, there is a reduction in all cell types in the blood and complete suppression of bone marrow. The disease is progressive and may be acute or chronic.

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Causes

  • The exact cause of aplastic anemia is unknown

  • It is thought to be possibly an autoimmune disease in which the the cells of the body react against its own cells.

  • High-dose radiation and chemotherapy treatments to fight cancer elsewhere in the body. Aplastic anemia can be a temporary side effect of these treatments.

  • Exposure to toxic chemicals such as some chemicals used in hair dyes, herbicides and insecticides. Exposure to benzene, which is an ingredient in gasoline, mothballs, paint, varnish removers, dry-cleaning solutions, some glues and household cleaners have also been associated to aplastic anemia.

  • Certain medications that are used to treat rheumatoid arthritis and some antibiotics.

  • An autoimmune disorder such as Systemic Lupus Erythematosus may involve stem cells in the bone marrow.

  • A viral infection of the bone marrow.

  • Rarely, aplastic anemia may occur in pregnancy.

  • Diseases that affect the bone marrow like leukemia and myelodysplastic disorders can eventually lead to aplastic anemia.

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Symptoms

Low Red Blood Cell count leads to:
Low White Blood Cell count causes an:
Low Platelet count results in bleeding tendencies like:
  • Fatigue and weakness.
  • Pallor
  • Shortness of breath on strenuous activity.
  • Rapid heart rate
  • Increased risk of infection.
  • Frequent or severe infections
  • Lymph nodes may be enlarged
  • Easy bruising
  • Nose bleeds
  • Bleeding gums
  • Prolonged bleeding from cuts.
  • Increased bleeding time

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Implications

Red blood cells play a very vital role of carrying oxygen from the lungs to all parts of the body. Without adequate numbers of red blood cells, the body's optimum demand for oxygen is not met and as a result, you feel fatigued.

In aplastic anemia, the bone marrow ceases to produce new blood cells. This translates in a shortage of all the cell types in the blood i.e. red blood cells which supply oxygen to different parts of the body, white blood cells that help to fight germs and platelets that help the blood to clot. Thus the risk of developing uncontrollable infections and bleeding rises tremendously.

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What You Can Do

  • Blood Transfusion
    Mild cases of aplastic anemia can be treated with blood transfusion and platelet transfusion that helps correct the low blood counts and relieve some of the symptoms.
  • Bone Marrow Transplant
    Severe aplastic anemia can be a life-threatening condition. Bone marrow transplant is indicated for younger patients with aplastic anemia.
  • Immuno-suppressants
    For those patients who do not have a matched bone marrow donor, antithymocyte globulin (ATG) is the alternative treatment of choice. ATG contains antibodies against human T cells and is used to suppress the body's immune system, allowing the bone marrow to resume its normal function of producing blood cells. Other immuno-suppressants such as cyclosporine may also be used for this purpose. Corticosteroids, such as methylprednisolone (Medrol, Solu-Medrol), are often given at the same time as these drugs to lessen their side effects.
  • Other Treatments
    If aplastic anemia does not respond to the above treatments, then it can be fatal. New treatment strategies are continually being studied and developed to treat the refractory form of this disease. Some of the new therapies include:
    • Growth Factors like granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF) and cytokines, may help stimulate the bone marrow to produce new blood cells especially white blood cells.
    • Male Hormones like androgens can stimulate blood cell production and may offer some benefit to people with mild to moderate aplastic anemia.
    • Stem Cell Transplants involves taking stem cells from the blood of a donor and injecting them into the patient with the hope that these stem cells would stimulate the production of healthy blood cells in the bone marrow.
    • Autologous Transplant of Stem Cells is a procedure in which stem cells are retrieved from the bone marrow of people with aplastic anemia. These stem cells are subjected to chemotherapy to destroy the diseased cells. The remaining healthy cells are then re-injected into the patient.
  • Prevention
    • Avoiding exposure to insecticides, herbicides, organic solvents, paint removers and other toxic chemicals that might lower your risk of developing this disease.
    • Avoid excessive exercise as anemia causes fatigue and shortness of breath. Long term anemia can damage your heart as the heart has to work harder to pump blood to compensate for a lack of oxygen in the blood.
    • Avoid sports and activities that may increase your risk of injury and bleeding.
    • Protect yourself from infections by avoiding contact with sick people.
  • Consult your physician immediately or go to the emergency room if there is spontaneous bleeding which is difficult to stop or if you notice frequent infections or unusual fatigue.

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Prognosis

Untreated aplastic anemia is an illness that leads to rapid death. Bone marrow transplantation has been successful in majority of young people, with long term survival of 80% whereas older people have a survival rate of 40 to 70%.

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Aplastic Anemia