Agranulocytosis

Agranulocytosis is a blood disorder in which the person has a low level of granulocytes. Granulocytes are a type of white blood cell. When they are examined under a microscope, they appear to contain granules, or small dark specks. Neutrophils, basophils, and eosinophils are all types of granulocytes. These cells are important in fighting infections.

What is going on in the body?

The bone marrow contains special cells known as stem cells. These are like infant cells that mature into many types of cells. Stem cells may develop into red blood cells, white blood cells, and platelets. Certain conditions can damage stem cells, or change their environment. The stem cells stop making blood cells. A low level of granulocytes in the body is called agranulocytosis. Since granulocytes are important in fighting infections, a person who has agranulocytosis is more prone to different types of infections.

What are the causes and risks of the condition?

Agranulocytosis can be caused by a number of factors, including:

autoimmune disorders, or conditions in which the body attacks its own tissues

chemotherapy

diseases that damage the bone marrow

hereditary disorders

radiation therapy

use of certain medications, such as clozapine or carbamazepine

What are the signs and symptoms of the condition?

Someone who has agranulocytosis may develop life-threatening or chronic infections. Symptoms vary, depending on the type of infection, but may include the following:

chills

fever

sore throat

ulcers in the mouth, stomach, or bowels

weakness

How is the condition diagnosed?

Diagnosis of agranulocytosis begins with a medical history and physical exam. The provider may order a complete blood count, or CBC, with a differential. This blood test measures the level of neutrophils, basophils, and eosinophils. Genetic testing may be done if heredity is suspected to be the cause.

If blood tests are abnormal, the provider may order a bone marrow biopsy. This test involves inserting a special needle into the hip bone. A sample is taken from the bone marrow.

What can be done to prevent the condition?

In most cases, agranulocytosis is not preventable.

What are the long-term effects of the condition?

Agranulocytosis increases a person’s risk for infections. If white bloodcell levels are extremely low, serious bacterial infections can occur. Thesecan include infections caused by bacteria that usually don’t cause any problemsin the body. Infection in the bloodstream, known as sepsis, may lead to life-threatening septic shock.

Someone who has agranulocytosis may develop severe lung infections. Strong antibiotics may or may not cure the infections. The individual is at risk for chronic infections. These can cause organ damage and scarring.

What are the risks to others?

Agranulocytosis is not contagious and poses no risk to others.

What are the treatments for the condition?

Initial treatment of agranulocytosis often includes the following:

antibiotics to treat or prevent infections

isolation to keep the person from contracting an infection

stopping the medication that is suspected as the cause of the agranulocytosis, which may resolve the problem

Other treatments may be ordered, depending on the cause of the agranulocytosis. These treatments include:

bone marrow transplant

granulocyte-colony-stimulating factors, such as GM-CSFs or G-CSFs, which stimulate the production of granulocytes

matched stem cell transfusions, which replace damaged stem cells

Bone marrow transplant is an effective treatment for some people with agranulocytosis. More than half of the good candidates for bone marrow transplant are cured. People are good candidates if they meet these criteria:

They are under the age of 40 years.

They have had good health prior to the transplant.

They have a matched donor, such as a family member.

What are the side effects of the treatments?

Medications used to treat agranulocytosis may increase the person’s risk for infection. Some may cause allergic reactions. A bone marrow transplant may cause bleeding, infection, or allergic reaction to anesthesia.

What happens after treatment for the condition?

Until blood cell counts return to normal, someone who has agranulocytosis is at risk for infection. It’s important to avoid risk factors, such as the following:

activities that cause a rapid heartbeat, chest pain, or shortness of breath

excessive exercise

exposure to contagious diseases

How is the condition monitored?

The person with agranulocytosis will have regular visits with the healthcare provider. Any new or worsening symptoms should be reported to the provider.