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Agranulocytosis

What is agranulocytosis?

Agranulocytosis is a condition where the absolute neutrophil count (ANC) is less than 100 per microliter of blood. Granulocytes are white blood cells that make up part of the immune system. Normally, there are at least 1500 per microliter of blood. They contain enzymes that can kill bacteria and other organisms and break down substances that may harm the body. Without granulocytes, one has a higher risk of developing frequent or chronic infections. Agranulocytosis is very serious and even life-threatening. Untreated, it can lead to death from the blood infection called septicemia.

Granulocytes are made up of three types of white blood cells. Neutrophils account for the largest share of the total number, followed by lymphocytes and monocytes. Basophils and eosinophils make up a smaller share. Each type of cell plays a role in the immune system’s ability to destroy harmful microorganisms.

For granulocyte counts between 100 and 1500 per microliter, the term “granulocytopenia”, which means too few granulocytes, is used. The lower the granulocyte count and the longer it remains low, the greater the risk of developing a dangerous infection.

Types of agranulocytosis

There are two main forms of agranulocytosis: acquired or inherited. If a person has the acquired form, either the bone marrow cannot produce enough stem cells that mature into granulocytes, or granulocytes are destroyed faster than they can be replaced. Usually, other cell types, such as red cells or platelets, are affected also.

The congenital or inherited form is due to a genetic abnormality and is usually present from birth.

What are the symptoms of agranulocytosis?

Symptoms may include:

  • sores in the mouth, throat, or gastrointestinal tract
  • chronic infections of the gums, throat, or skin
  • fever
  • chills

How common is agranulocytosis?

Agranulocytosis occurs among all age groups and in all racial groups. The acquired form is more common among elderly people. The inherited form is more often seen in children. The exact frequency of the condition is not known, but the number of cases is estimated at 1 million to 3.4 million people per year.

What causes agranulocytosis?

There are many different causes of acquired agranulocytosis. These include:

  • chemotherapy to treat cancer
  • the use of some prescription drugs, particularly certain antibiotics and anti-psychotics
  • exposure to toxic substances, such as arsenic or mercury
  • bone marrow failure for other reasons
  • other medical conditions like tumors or some autoimmune diseases

The congenital (inherited) form of agranulocytosis is caused by a genetic abnormality. Infantile genetic agranulocytosis (Kostmann) disease is a rare form of the inherited disease that is present in newborns.

How is agranulocytosis diagnosed?

Your doctor will ask about your medical history and general health. He or she will perform a physical examination. The doctor is likely to order a complete blood count (CBC) to determine the level of granulocytes in your blood. If the results of the CBC are abnormal or not clear, a bone marrow biopsy might be performed. During the biopsy, a small amount of bone marrow is removed from the hip bone using a needle. The doctor might order a genetic test if he or she suspects the inherited form.

How is agranulocytosis treated?

Antibiotics—These drugs often are used to prevent bacterial infections.

Stopping medication thought to cause the disease—The doctor may tell you to stop taking the drug for a while to see if you improve. Recovery usually occurs within 10 to 14 days. A different medication may be substituted for the original. If there is no substitute, and your condition is not severe, you might be told to take the drug again while you are monitored closely by your doctor.

Granulocyte colony-stimulating factor—If other methods do not work, you might need to take shots of a hormone that stimulates the bone marrow to produce more granulocytes. This hormone is known as granulocyte colony-stimulating factor (G-CSF) and is made by the body. There is also a synthetic version. Studies show that G-CSF can reduce the seriousness of neutropenia (low number of neutrophils) in patients with some cancers. Three forms of G-CSF are available: Neupogen® (filgrastim), Neulasta® (pegfilgrastim, a long acting form of filgrastim), and Granocyte® (lenograstim).

Immune suppression----When an autoimmune cause is suspected, immune suppressing medications such as prednisone may be used.

Bone marrow transplants—For cases that do not respond to other types of treatment, a bone marrow transplant may be needed if a donor can be found. Bone marrow transplants generally yield the best outcomes for patients who are under 40 years old and are in good health.

General measures----Avoid contact with people who have infections and stay away from crowds. Avoid fruits and vegetable which cannot be peeled. Avoid cut flowers or working in the soil.

How can agranulocytosis be prevented?

There is no way to prevent this disease.

References

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 4/19/2013…#15262

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