What is agranulocytosis?

Granulocytes, also known as neutrophils, are white blood cells that make up part of a person’s immune system. These cells contain enzymes that can kill bacteria and other organisms and break down substances that may harm the body. Without granulocytes, a person has a higher risk of developing frequent or chronic (long-term) infections.

Agranulocytosis (lack of granulocytes) is a condition in which the absolute neutrophil count (ANC) is less than 100 neutrophils per microliter of blood. Normally, there are at least 1500 neutrophils per microliter of blood.

For granulocyte (neutrophil) counts between 100 and 1500 per microliter of blood, the term “granulocytopenia” or “neutropenia” is used. This means that too few neutrophils are present. The lower the neutrophil count and the longer it remains low, the greater the risk of developing a dangerous infection.

Agranulocytosis is very serious and can be life-threatening. If it is not treated, it can lead to death, most commonly through septicemia (bacterial infection of the blood).

What are the symptoms of agranulocytosis?

Symptoms of agranulocytosis may include:

  • Sores in the mouth, throat, or gastrointestinal tract
  • Chronic infections of the gums, throat, or skin
  • Fever
  • Chills
  • A life-threatening drop in blood pressure, which may cause weakness or lightheadedness

What causes agranulocytosis?

Agranulocytosis may be acquired or inherited. In the acquired form, either the bone marrow cannot produce enough stem cells that mature into granulocytes, or granulocytes are destroyed more quickly than they can be replaced. Usually, other cell types, such as red cells or platelets, are affected, also.

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-thyroid and anti-psychotic medications
  • Exposure to toxic substances, such as arsenic or mercury
  • Bone marrow failure disorders such as aplastic anemia
  • Other medical conditions, such as tumors or some autoimmune diseases

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

How common is agranulocytosis?

Agranulocytosis occurs among all age groups and in all racial groups. The acquired form is more common among older people, while 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.

How is agranulocytosis diagnosed?

Your doctor will ask about your medical history and general health, and 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 recommended. During the biopsy, a small amount of bone marrow is removed from the hip bone with a needle. The doctor might order a genetic test if he or she suspects the inherited form.

How is agranulocytosis treated?

Agranulocytosis can be treated in a number of ways:

  • Stopping a medication that is believed 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 your doctor watches you closely.
  • Antibiotics: These drugs may be used to prevent bacterial infections, and are used to treat infections that have developed.
  • 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 (man-made) 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: filgrastim (Neupogen®), pegfilgrastim, a long-acting form of filgrastim (Neulasta®), and lenograstim (Granocyte®).
  • 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 in good health.
  • General measures: People with agranulocytosis should stay away from crowds. They should also avoid: contact with people who have infections; fruits and vegetables that cannot be peeled, and; cut flowers or working in the soil.

How can agranulocytosis be prevented?

Agranulocytosis cannot be prevented. The goal is to find out why agranulocytosis has developed and recommend appropriate treatment.

References

© Copyright 1995-2017 The Cleveland Clinic Foundation. All rights reserved.

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: 2/2/2017…#15262