Agranulocytosis
En Español (Spanish Version)

Definition
Agranulocytosis is a low level of white blood cells. These blood cells are part of the immune system. They help fight infections.

Agranulocytosis may be:

  • Acquired—Develops after medical treatment or specific drugs. May appear suddenly or develop over time.
  • Congenital—present at birth
    • Cyclic neutropenia—cycles up and down over a 21-day period
    • Severe congenital neutropenia
White Blood Cells

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Causes
Agranulocytosis is caused by destruction of white blood cells or by the failure of bone marrow to make enough white blood cells.

With congenital agranulocytosis, these problems are caused by a genetic defect.

With acquired agranulocytosis, these problems may be caused by:

  • Infections by virus, bacteria, or parasite
  • Underlying inflammatory condition
  • Chemotherapy
  • Drugs—used in medical treatment or recreational use
  • Autoimmune disease—your immune systems attacks your own tissue such as white blood cells
  • Damage to bone marrow usually by chemicals, radiation, or cancers
  • Certain toxins
  • Poor nutrition—particularly low protein intake
Risk Factors
Factors that increase your chance of developing agranulocytosis include:

  • Undergoing chemotherapy treatment for cancer
  • Taking certain medications, including some antithyroid medication, antidepressants, antihistamines, and anticonvulsants
  • Infection
  • Exposure to certain chemical toxins or radiation
  • Autoimmune diseases
  • Enlargement of the spleen
  • Vitamin B-12 or folate deficiency
  • Leukemia or myelodysplastic syndromes
  • Aplastic anemia or other diseases of the bone marrow
  • Family history of certain genetic diseases
Symptoms
Symptoms of agranulocytosis may include:

Symptoms of congenital agranulocytosis may include:

  • Mild infections of skin, mouth, and nose
  • Poor weight gain in children
Diagnosis
Your doctor will ask about your symptoms and medical history. You will be asked about recent infections, medical treatments, and medications. A physical exam will be done.

Your bodily fluids and tissues may be tested. This can be done with:

  • Blood tests
  • Bone marrow test
  • Urine tests
Treatment
Treatment will be based on the type and cause of agranulocytosis that you have. Options include the following:

Leukocyte Transfusion
Leukocytes are a type of white blood cell. These cells are collected from a donor and carefully screened. They are then delivered through an IV. These white blood cells may make up for the deficit caused by agranulocytosis.

Medication for Infections Treatment
Antiviral, antibiotic, and antifungal medication may be needed to:

  • Treat an infection that could be causing agranulocytosis
  • Treat an infection that resulted from agranulocytosis
  • Prevent an infection in people at high risk—this may include people with cancer or immune disorders
White Blood Cell-stimulating Factors
Granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF) encourages the body to make more white blood cells. This may help with certain types of agranulocytosis.

Remove Causative Agent
When possible, the toxin or drug that is causing the problems will be removed.

Prevention
Your doctor will monitor you if you are taking medication or having medical treatment that could lead to agranulocytosis. You may be given white blood cell stimulating medications before having treatments. This may prevent agranulocytosis.




RESOURCES:
American Dental Association

National Organization for Rare Disorders, Inc.

CANADIAN RESOURCES:
The College of Family Physicians of Canada


References:
Boulton F, Cooper C, et al. Neutropenia and agranulocytosis in England and Wales: incidence and risk factors. American Journal of Hepatology. 2003 Apr;72(4):248-54.

Neutropenia. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated July 11, 2013. Accessed August 20, 2013.

Last Reviewed August 2013



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