Agranulocytosis
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Agranulocytosis

(Granulocytopenia; Granulopenia; Neutropenia)

Pronounced: ay-GRAN-yoo-loh-cy-TOH-sis

En Español (Spanish Version)

Definition

Agranulocytosis is a condition that results from failure of an individual's bone marrow to produce a sufficient quantity of white blood cells, or increased destruction of the white blood cells. As a result, the white blood cell count will be low. Acquired agranulocytosis occurs most often as a result of medications or treatments. A congenital agranulocytosis is a condition that someone is born with. Agranulocytosis usually responds well to treatment, so contact your doctor if you think you may have this condition.

White Blood Cells

White Blood Cells

© 2008 Nucleus Medical Art, Inc.

Causes

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

  • Congenital genetic abnormality
  • Tumors, infections or other inflammations, or fibrosis of the bone marrow
  • Toxins, autoimmune diseases, infections, and other causes
  • Aplastic anemia
  • Certain medications or treatments

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. The following factors increase your chances of developing agranulocytosis. If you have any of these, tell your doctor:

  • Undergoing chemotherapy treatment for cancer
  • Taking certain drugs
  • 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

If you experience any of these symptoms do not assume it is due to agranulocytosis. These symptoms may be caused by other, less serious health conditions. If you experience any one of them, see your physician.

  • Rapid onset of fever, chills, jaundice , weakness, or sore throat
  • Bacterial pneumonia
  • Ulcers in the mouth
  • Bleeding gums
  • Low white blood cell count
  • Infections, including fungal

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include the following:

  • Blood test to determine white blood cell count
  • Urine or other fluids may be tested for infectious agents if the person has a fever
  • Bone marrow test ( biopsy and aspiration)
  • Genetic tests may be needed in some people
  • Antineutrophil antibodies may need to be tested for in people with autoimmune disease

Treatment

Talk with your doctor about the best treatment plan for you. Treatment options include the following:

Leukocyte Transfusion

Transfusion of white blood cells to replace deficit may be of benefit in certain people.

Antibiotic Treatment

Used to treat infection that could be causing agranulocytosis or resulting from agranulocytosis.

White Blood Cell-stimulating Factors

Depending on the cause, some people may benefit from treatments with granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF).

Remove Causative Agent

Remove toxin/drug or treat primary disorder.

Prevention

To help reduce your chances of getting agranulocytosis, take the following steps:

  • Talk to your doctor about preventive measures when receiving white blood cell-reducing therapies.
    • White blood cell-stimulating treatments such as G-CSF or GM-CSF
    • Treatments to prevent the loss of white blood cells

RESOURCES:

Marquette University School of Dentistry
http://www.dental.mu.edu/oralpath/lesions/agranulocytosis/agranulocytosis.htm

National Organization for Rare Disorders, Inc.
http://www.rarediseases.org/

CANADIAN RESOURCES:

British Columbia Ministry of Health
http://www.bchealthguide.org/kbase/nord/nord209.htm

Canadian Family Physician
http://www.cfpc.ca/cfp/

References:


Boulton F, Cooper C, Hagenbeek A, et al. Neutropenia and agranulocytosis in England and Wales: incidence and risk factors. American Journal of Hepatology . 2003 Apr;72(4):248-54. PubMed website. Available at: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12666135 . Accessed December 18, 2006.

Hoffman R. et al. Hematology: Basic Principles and Practice .4th ed. Philadelphia: Elsevier, 2005.

Mandell GL et al. Principles and Practice of Infectious Diseases . 6th ed. London: Churchill Livingstone, 2005.

Tajiri J, Noguchi S, MurakamiT, Murakami N. Antithyroid drug-induced agranulocytosis. The usefulness of routine white blood cell count monitoring. Arch Intern Med . 1990 Mar 1;150(3). Archives of Internal Medicine website. Available at: http://archinte.ama-assn.org/cgi/content/abstract/150/3/621 . Accessed December 18, 2006.

Tyson MC, Rosenthal N, Vogel P. The value of penicillin in the treatment of agranulocytosis caused by thiouracil. Blood . 1946;1(1):53-66. Blood Journal website. Available at: http://www.bloodjournal.org/cgi/content/abstract/1/1/53 . Accessed December 18, 2006.



Last reviewed February 2008 by Rosalyn Carson-DeWitt, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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