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Agranulocytosis

Alternate Names

  • malignant neutropenia

Definition

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.

Risks

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 (i.e., Clozaril, FazaClo ODT) or carbamazepine (i.e., Carbatrol, Equetro, Tegretol)

Prevention

What can be done to prevent the condition?

In most cases, agranulocytosis is not preventable.

Diagnosed

How is the condition diagnosed?

Diagnosis of agranulocytosis begins with a medical history and physical exam. The professional 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 professional 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.

Long Term Effects

What are the long-term effects of the condition?

Agranulocytosis increases a person's risk for 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. 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.

Other Risks

What are the risks to others?

Agranulocytosis is not contagious and poses no risk to others.

Treatments

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 for bone marrow transplant 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.

Side Effects

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 a reaction to anesthesia.

After Treatment

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

Monitor

How is the condition monitored?

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

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