Agranulocytosis

Written by Helen Colledge and Matthew Solan | Published on July 18, 2012
Medically Reviewed by Brenda B. Spriggs, MD, MPH, FACP

Overview

Agranulocytosis is a rare condition that occurs when the bone marrow does not make enough neutrophils, the white blood cells needed to fight infections. Neutrophils make up the largest portion of white blood cells in the body. Neutrophils are a critical part of the body’s immune system. They are often the first immune cells to arrive at the site of any infection, where they consume and destroy harmful invaders like bacteria.

Agranulocytosis can turn minor infections into serious ones. Weak microbes or tiny organisms that usually cause no harm suddenly can become strong enough to attack the body.

What Are The Types and Causes Of Agranulocytosis?

Agranulocytosis can be congenital, which means you are born with the condition, or it can be acquired from certain drugs and medical procedures.

Acquired forms of agranulocytosis can be caused by:

  • drugs such as clozapine and antithyroid medication
  • exposure to chemicals like the insecticide DDT
  • diseases that affect the bone marrow, such as cancer
  • infections
  • exposure to radiation
  • autoimmune diseases such as systemic lupus erythematosus
  • nutritional deficiencies including low B12 and folate
  • bone marrow transplants
  • blood transfusions
  • chemotherapy

What Are The Risk Factors?

Women are more likely to develop agranulocytosis. Although it can occur at any age, inherited forms of the condition are more often found in children. Acquired agranulocytosis occurs most frequently in elderly people.

What Are The Symptoms Of Agranulocytosis?

The early symptoms of agranulocytosis include a sudden fever, chills, a sore throat, and weakness in the limbs. The mouth and gums may be sore, mouth ulcers can develop, and gums might begin to bleed.

Other signs of agranulocytosis can include:

  • fast heart rate
  • rapid breathing
  • low blood pressure
  • skin abscesses

How Is Agranulocytosis Diagnosed and Tested?

A detailed history must include questions about recent drug treatments or illnesses. Blood and urine samples are needed to check for infection and white blood cell counts. If a problem with the bone marrow is suspected, a marrow sample may be taken.

Genetic testing will be necessary to check for a hereditary form of agranulocytosis. Also, tests for possible autoimmune disorders may be required.

Treatment Options for Agranulocytosis

If agranulocytosis has arisen from an underlying illness, then that condition will be treated first.

If the agranulocytosis is caused by a drug needed for another condition, a substitute treatment will be offered. For anyone taking several different drugs, it may be necessary to stop taking them. This could be the only way to find which one(s) may be the cause of the problem.

Antibiotics or antifungal drugs may be given to treat any infections.

For some people, such as those who have acquired agranulocytosis from chemotherapy, a treatment called a colony-stimulating factor can be used. This treatment encourages the bone marrow to produce more neutrophils and can be used along with the chemotherapy cycles.

Although not widely used, a transfusion of neutrophils may be the best treatment.

What Is The Long-Term Outlook For Agranulocytosis?

Without treatment, agranulocytosis can lead to death by infection. However, if you receive appropriate and timely treatment, the outlook is positive. People who develop agranulocytosis following a viral infection often find the condition resolves itself and the prognosis is good.

Is There A Way To Prevent It?

The only way to prevent agranulocytosis is to avoid any drugs that may cause it. However, if a drug known to trigger the condition must be taken, regular blood tests to check neutrophil levels will be required. If lowered neutrophil counts are noted, the drug should be discontinued.

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