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Thursday, April 30, 2015

Pancytopenia

Pancytopenia

Pancytopenia is a deficiency of all types of blood cells, including white blood cells, red blood cells, and platelets. It occurs when your body cannot produce enough blood cells because the bone marrow stem cells that form blood cells do not function normally. Pancytopenia has widespread effects on the entire body by leading to oxygen shortage as well as problems with immune function. A plastic anemia is a medical term that refers to a decrease in production of all types of blood cells.
Pancytopenia occurs in two forms: idiopathic, in which the cause is not known, but is often autoimmune, meaning that the body attacks its own tissues as foreign substances; and secondary, often caused by environmental factors. Approximately half of all pancytopenia cases are idiopathic. In other cases, viral infections, radiation or chemotherapy treatments, drug reactions, and exposure to toxins may precipitate the development of pancytopenia.

Pancytopenia may develop slowly over time or suddenly, and it can progress in a variety of ways.

causes

Pancytopenia can be caused by heredity, medications, or exposure to environmental contaminants such as radiation or arsenic. In approximately half of cases, called idiopathic cases, the exact cause of the pancytopenia is not known. It may be linked to an autoimmune disorder, in which the body’s immune system attacks its own tissues as foreign substances, or an environmental contaminant. In rare cases, pregnancy can lead to autoimmune processes that may trigger Pancytopenia
It is important for your health care professional to determine the cause of pancytopenia in order to recommend the appropriate treatment. For example, pancytopenia caused by an environmental contaminant may spontaneously resolve when the contaminant is removed.

Environmental causes of pancytopenia

Environmental factors, such as medications, toxins or infections, have been identified as causes of pancytopenia in some cases. Examples include:
  • Certain medications, including some antibiotics and immunosuppressant drugs
  • Chemical toxins such as benzene              
  •   Chemotherapy
  • Radiation exposure                                 
  •      Viral infections
Signs and symptoms

You may experience pancytopenia symptoms daily or just once in a while. At times, any of these symptoms can be severe:
  • Bleeding gums                                 
  •  Fatigue
  • Frequent infections                            
  •  Nosebleeds
  • Pale skin or pallor                            
  •  Rapid heart rate (tachycardia)
  • Rash                                                      
  •   Shortness of breath
  • Skin discoloration such as bruising       
  •  Weakness (loss of strength)

Serious symptoms that might indicate a life-threatening condition

  • Confusion or loss of consciousness for even a brief moment
  • Excessive bleeding without apparent cause
  • Extreme fatigue, weakness, or shortness of breath
  • High fever (higher than 101 degrees Fahrenheit)

Reducing your risk of pancytopenia

Although pancytopenia is unavoidable in many cases, you may be able to lower your risk of pancytopenia by:
  • Avoiding environmental contaminants (for example, benzene or arsenic)
  • Avoiding radiation exposure

Treatment

In very mild cases of pancytopenia, treatment may not be necessary. In moderate cases, blood transfusions may help restore blood cell counts; however, transfusions may become less effective over time. In severe cases, treatments such as bone marrow transplant and stem cell therapy may be required to restore the ability of bone marrow to produce blood cells. Such treatments are generally effective in younger patients, but older patients may also require the use of immunosuppressant drugs or drugs that stimulate the bone marrow
In cases related to environmental factors, pancytopenia may resolve on its own when the precipitating factor is removed or the underlying condition is treated.

Immunosuppressant medications used to treat pancytopenia

If the immune system is suspected of attacking bone marrow, immunosuppressant drugs may be administered. Examples include:
  • Antithymocyte antibodies (Thymoglobulin), which suppress the body’s natural immune response
  • Corticosteroids, such as methylprednisolone (Medrol, Solu-Medrol)
  • Cyclophosphamide (Cytoxan)
  • Cyclosporine (Sand immune, Neural)

Bone marrow–stimulating drugs used to treat pancytopenia

Sometimes, drugs that stimulate bone marrow function are prescribed. Examples include:
  • Epoetin alfa (Epogen, Procrit)                  Filgrastim (Neupogen)
  • Pegfilgrastim (Neulasta)                           Sargramostim (Leukine, Prokine)

What are the potential complications of pancytopenia?

Left untreated, pancytopenia is extremely serious and can lead to life-threatening bleeding and infections. In younger patients, treatments such as bone marrow transplant and blood transfusion are generally successful at treating pancytopenia, but complications tend to be more severe in the elderly. You can help minimize your risk of serious complications by following the treatment plan you and your health care professional design specifically for you.
Complications of pancytopenia or its treatment include:
  • Bleeding in the brain
  • Complications from blood transfusions
  • Complications from medications used to treat the condition
  • Poor reaction to bone marrow transplant (graft rejection)
  • Sepsis (life-threatening bacterial blood infection)
  • Severe bleeding



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