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.
Without granulocytes, one has a higher risk of developing frequent or chronic infections. Agranulocytosis is very serious and even life-threatening. Untreated, it can lead to death from the blood infection called septicemia.
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.
Types of agranulocytosis
There are two main forms of agranulocytosis: acquired or inherited. If a person has the acquired form, either the bone marrow cannot produce enough stem cells that mature into granulocytes, or granulocytes are destroyed faster than they can be replaced. Usually, other cell types, such as red cells or platelets, are affected also.
The congenital or inherited form is due to a genetic abnormality and is usually present from birth.
Causes of Agranulocytosis
Agranulocytosis is caused by the 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
- 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
Agranulocytosis results in a person not having enough of a specific type of white blood cells, called neutrophils or granulocytes. A low neutrophil count (neutropenia) may also occur when white blood cells are destroyed faster than they can be produced.
Risk Factors of Agranulocytosis
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
- 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
How common is agranulocytosis?
Agranulocytosis occurs among all age groups and in all racial groups. The acquired form is more common among elderly people. The inherited form is more often seen in children. The exact frequency of the condition is not known, but the number of cases is estimated at 1 million to 3.4 million people per year.
What Are The Signs and 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?
Your doctor will ask about your medical history and general health. He or she will perform a physical examination. The doctor is likely to order a complete blood count (CBC) to determine the level of granulocytes in your blood. If the results of the CBC are abnormal or not clear, a bone marrow biopsy might be performed. During the biopsy, a small amount of bone marrow is removed from the hip bone using a needle. The doctor might order a genetic test if he or she suspects the inherited form.
How is agranulocytosis treated?
These drugs often are used to prevent bacterial infections.
Stopping medication thought to cause the disease
The doctor may tell you to stop taking the drug for a while to see if you improve. Recovery usually occurs within 10 to 14 days. A different medication may be substituted for the original. If there is no substitute, and your condition is not severe, you might be told to take the drug again while you are monitored closely by your doctor.
Granulocyte colony-stimulating factor
If other methods do not work, you might need to take shots of a hormone that stimulates the bone marrow to produce more granulocytes. This hormone is known as granulocyte colony-stimulating factor (G-CSF) and is made by the body. There is also a synthetic version. Studies show that G-CSF can reduce the seriousness of neutropenia (low number of neutrophils) in patients with some cancers. Three forms of G-CSF are available: Neupogen® (filgrastim), Neulasta® (pegfilgrastim, a long acting form of filgrastim), and Granocyte® (lenograstim).
When an autoimmune cause is suspected, immune suppressing medications such as prednisone may be used.
Bone marrow transplants
For cases that do not respond to other types of treatment, a bone marrow transplant may be needed if a donor can be found. Bone marrow transplants generally yield the best outcomes for patients who are under 40 years old and are in good health.
Avoid contact with people who have infections and stay away from crowds. Avoid fruits and vegetable which cannot be peeled. Avoid cut flowers or working in the soil.
How to Prevent Agranulocytosis
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
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.