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agranulocytosis

Alternative Names 
severe neutropenia

Definition
Agranulocytosis (ay-gran-you-lo-site-oh-sis) is a condition caused by a drop in a certain type of white blood cells in the body.

What is going on in the body? 
White blood cells fight infection. They are made in the bone marrow, the inside part of bones where many types of blood cells are made. White blood cells travel in the bloodstream. These blood cells can be low when the bone marrow does not make enough of them. Agranulocytosis can also be caused by conditions that cause the break down of these cells in the bloodstream.

This condition puts people at risk for developing infections. If white blood cell levels are extremely low, serious bacterial infections can occur. These can include infections caused by bacteria that usually don't cause any problems in the body. These infections may develop in the bloodstream and can be very dangerous.

What are the causes and risks of the disease? 
There are many causes of this disorder. Some are hereditary. Chemotherapy or radiation treatment for cancer can cause temporary or permanent agranulocytosis. Autoimmune diseases, or diseases in which the body attacks its own tissues, can also cause the condition. Other causes include vitamin B12 or folic acid deficiency and diseases that damage the bone marrow.

What can be done to prevent the disease? 
This disease can be caused by some medications including those used for chemotherapy. Rarely a medication called carbamazepine can cause agranulocytosis. This medication is most often used for seizures or epilepsy.

How is the disease diagnosed? 
A full blood count is needed to make a diagnosis. The cause can be found by testing the bone marrow. Genetic testing may be done if heredity is suspected to be the cause.

What are the long-term effects of the disease? 
Severe lung infections can develop from this disease. Strong antibiotics may or may not cure the infections.

Chronic infections can also develop, as well as life-threatening infections. These can cause repeated hospitalisations, surgery, organ damage, and scarring.

What are the risks to others? 
Agranulocytosis is not a contagious disease.

What are the treatments for the disease? 
People who have this condition and a fever are treated with strong antibiotics to help cure the infections. Often people are given strong antibiotics even if the site of the infection cannot be found.

Hereditary causes may be hard to treat.

Transfusions of white blood cells have been tried, but have not been successful. Bone marrow transplants or stem cell infusions are experimental treatments. Vitamin deficiencies are easily treated.

Medications can be used to stimulate the bone marrow to make more white blood cells. But these treatments are often ineffective.

What are the side effects of the treatments? 
Different medication treatments have different side effects. Some side effects may be:
  • Cushing's syndrome
  • increased blood sugar
  • cataracts
  • easy bruising
  • ulcers
  • bone loss
  • infections
Allergic reactions can occur.

What happens after treatment for the disease? 
Frequent blood tests are necessary for people with this disease. The problem may recur if the cause is a medication that is necessary (as in the case of chemotherapy). Also, the condition may recur if it is caused by heredity.

How is the disease monitored? 
Monitoring is done by taking a small amount of blood and doing a test called a full blood count. This test gives information about the number of white blood cells in the blood. A bone marrow biopsy may also be needed.

Author: Thomas Fisher, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request
 


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