Agranulocytosis

What is agranulocytosis?

Agranulocytosis is a condition where the bone marrow cannot produce in enough quantity, a specific type of white blood cells called neutrophils. They are the largest type of white blood cells in the body.

Neutrophils are important elements of the immune system that are involved in fighting infections in the body. In fact, they are known to be the first immune cells that will get to an infection site before any other disease-fighting cell gets there. They will devour any invading microorganism in sight. In the disorder, neutrophils levels are very low hence a common mild infection can become really serious.

The disorder is a rare medical condition.

What are the symptoms of agranulocytosis?

This disorder is most times is asymptomatic. It has some symptoms that occur very early and they are:

  • sudden fever
  • bleeding gums
  • chills
  • weak limbs
  • sore throat
  • sore mouth
  • mouth ulcers

Other signs and symptoms of the disorder can include:

  • low blood pressure
  • skin abscesses
  • increased heart rate
  • rapid breathing

Agranulocytosis
Photo Credit: Medical News Today

What are the types and causes of agranulocytosis?

Two shades of agranulocytosis that exist are congenital and acquired agranulocytosis.

In congenital, you’re born with the condition but in acquired, you ‘acquire’ it. That was a funny one from me, right?

Alright, just know that when you have acquired agranulocytosis, you developed it much later in life either because you took a drug or you underwent a medical procedure that affected your bone marrow. Whichever the shade of agranulocytosis, what you should know is, neutrophils will be very low in the body of affected persons.

About 1,500 to 8,000 neutrophils per microliter (mcL) of blood is the normal in healthy adults. But you know what? If you have agranuloctytosis, you won’t have up to 500 neutrophils per mcL.

Now let me tell you a few things about the acquired form of agranulocytosis. In acquired agranulocytosis, your bone marrow fails to make enough neutrophils available in the body.

This could mean that the bone marrow produce neutrophils but they end up not growing into maturity. Or the bone marrow could have actually produced enough neutrophils but something happen along the line and they get destroyed.

Congenital agranulocytosis is inherited from parents that have the gene.

The different ways acquired granulocytosis can be developed include:

  • chemotherapy
  • exposure to chemicals i.e. insecticide DDT
  • nutritional deficiencies i.e. vitamin B-12 and folate deficiency
  • certain medications
  • bone marrow diseases
  • radiation exposure
  • serious infections
  • systemic lupus erythematosus and other autoimmune diseases

A 1996 study showed that close to 70% cases of acquired agranulocytosis were medications-induced. Some of the drugs that can induce agranulocytosis are:

  • antithyroid medications i.e. carbimazole and methimazole (aka Tapazole)
  • anti-inflammatory i.e. sulfasalazine (aka Azulfidine), dipyrone (aka Metamizole), and nonsteroidal anti-inflammatory drugs (NSAIDs)
  • antipsychotics i.e. clozapine (aka Clozaril)
  • antimalarials i.e. quinine

What are the risk factors of agranulocytosis?

The disorder has no age limitations. It is more common in women than in men. Whilst the acquired form of agranulocytosis is more seen amongst older adults, the congenital forms will be prevalent amongst children. Children who have agranulocytosis might not live beyond their childhood years.

How is agranulocytosis diagnosed?

The first thing your doctor will do will be to take your history in detail. Questions that border around your recent medication use, illness etc. will be asked you.

Also, your blood and urine will be taken for test and analysis. Your neutrophils will be counted in the blood sample collected.

You might have to undergo a genetic test, too, so as to find out whether you have the congenital form of agranulocytosis. The test will also find out if there are any autoimmune diseases present in your body.

Treatment options for agranulocytosis

If the disorder was developed because there is an underlying illness, that illness will have to be handled first. If a drug was the cause, you will have to discontinue using that drug and get an alternative for it.

Antibiotics or antifungal drugs will be given to you to take care of the infection.

If you have the acquired form of the disorder because of chemotherapy you underwent, your doctor will likely administer a colony-stimulating factor (CSF). With CSF, your bone marrow will be induced to increase its production of neutrophils.

Neutrophils transfusion is another approach to helping you. But it is not that common.

What is the long-term outlook for agranulocytosis?

This disorder, irrespective of the form, predisposes you to frequent attacks and infections.

You therefore have to treat as important, your treatment.

Sepsis is the most common complication that can arise from the disorder. What sepsis simply means is that an infection has spread through all your body tissues. Sepsis can kill.

But if you get medical attention on time, you can save yourself from any complication that agranulocytosis can present. There are some claims that the disorder can resolve on its own after the occurrence of a viral infection.

Is there a way to prevent agranulocytosis?

To avoid developing the disorder, avoid taking medications that can induce it. Some of these drugs have already been listed in this article. It will also make much sense if you can get more information from your doctor on other drugs or therapies that can induce the disorder. If you must take a medication that can induce low blood count of neutrophils, for instance, your doctor will give you instructions as well as monitor you within the period of time when you are on the medication.

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