Agranulocytosis: Symptoms, Types, Causes, Risk Factors, Diagnosis, Treatment, Outlook and Prevention

It is a rare condition in which the bone marrow does not produce a certain type of white blood cell, most often neutrophils.

Neutrophils are a type of white blood cell that your body needs to fight infection. They make up the highest percentage of white blood cells in your body.

Neutrophils are a critical part of your body’s immune system . They are often the first immune cells to reach the site of infection. They consume and destroy harmful invaders like bacteria.

In agranulocytosis, the low level of neutrophils means that even minor infections can progress to serious.

Weak microbes or germs that usually do not cause sudden damage can evade the body’s defenses to attack your body.

What are the symptoms of agranulocytosis?

Agranulocytosis can sometimes be asymptomatic in the absence of an infection. The early symptoms of agranulocytosis may include:

  • Sudden fever
  • A cold.
  • Throat pain.
  • Weakness in your limbs
  • Mouth and gum pain.
  • Ulcers in the mouth
  • Bleeding gums

Other signs and symptoms of agranulocytosis can include:

Types and causes

There are two types of agranulocytosis, the first type is congenital, which means that you are born with the condition. The second type is acquired. You can get agranulocytosis from certain medications or medical procedures.

In both forms of agranulocytosis, you have a dangerously low neutrophil count.

Healthy levels in adults generally fall in the range of 1,500 to 8,000 neutrophils per microliter (mcL) of blood. In agranulocytosis, it has less than 500 per mcL.

In acquired agranulocytosis, something causes the bone marrow to not make neutrophils or to produce neutrophils that do not develop into fully mature, functional cells.

It’s also possible that something is causing the neutrophils to die too quickly. In congenital agranulocytosis, you inherit a genetic abnormality that causes it.

Acquired granulocytosis can be caused by:

  • Certain medications
  • Exposure to chemicals, such as the insecticide DDT.
  • Diseases that affect the bone marrow, such as cancer.
  • Serious infections
  • Exposure to radiation.
  • Autoimmune diseases, such as systemic lupus erythematosus .
  • Nutritional deficiencies, including low levels of vitamin B-12 and folic acid.
  • Chemotherapy.

A 1996 study found that about 70 percent of acquired agranulocytosis cases are drug-related. Medications that can cause agranulocytosis include:

  • Antithyroid medications, such as carbimazole and methimazole (Tapazol).
  • Anti-inflammatory drugs, such as sulfasalazine (Azulfidine), dipyrone (Metamizole), and non-steroidal anti-inflammatory drugs (NSAIDs).
  • Antipsychotics such as clozapine (Clozaril).
  • Antimalarials, such as quinine.

What are the risk factors for agranulocytosis?

Women are more likely to develop agranulocytosis than men. It can happen at any age.

However, inherited forms of the condition are most often found in children, who generally pass out of this condition long before reaching adulthood.

Acquired agranulocytosis occurs most often in older adults.

How is it diagnosed?

Your doctor will likely take a detailed medical history. This includes questions about recent illnesses or treatments.

Blood and urine samples are needed to detect infection and to do a test known as a white blood cell count. Your doctor may take a marrow sample if he suspects there is a problem with your bone marrow.

Genetic testing is necessary to detect an inherited form of agranulocytosis. You may need to be tested for possible autoimmune disorders.

Treatment options for agranulocytosis

If agranulocytosis is due to an underlying disease, that condition will be treated first.

If a drug needed for another condition causes agranulocytosis, your doctor may prescribe a replacement treatment. If you take several different medications, you may need to stop taking them.

This might be the only way to find out which medicine is causing the problem. Your doctor will likely prescribe antibiotics or antifungal medications to treat any infection.

A treatment called colony-stimulating factor may be used for some people, such as those who have acquired agranulocytosis from chemotherapy.

This treatment encourages the bone marrow to make more neutrophils. It can be used in conjunction with your chemotherapy cycles.

Although not widely used, a neutrophil transfusion may be the best temporary treatment for some people.

What is the long-term outlook?

Because agranulocytosis makes you vulnerable to infection, it can be very dangerous if left untreated. A complication of agranulocytosis is sepsis. Sepsis is an infection of the blood. Without treatment, sepsis can be fatal.

With prompt treatment, the outlook for agranulocytosis is better. In many cases, the condition can be managed.

People who develop agranulocytosis after a viral infection may even find that the condition resolves on its own.

Is there a way to prevent agranulocytosis?

The only way to prevent agranulocytosis is to avoid the drugs that can cause it.

If you need to take a drug known to trigger the condition, you will need to have regular blood tests to monitor your neutrophil levels.

Your doctor may advise you to stop taking the medicine if your neutrophil count has decreased.