Are your white blood cells dangerously low? Is that agranulocytosis? Let's talk about it!

Do you get sick all the time? Do you feel weak and tired even for a little while? Sometimes this can be due to a dangerous decrease in your body's defenses, namely neutrophils, a type of white blood cell. Today we are talking about a serious but treatable condition called agranulocytosis . Don't worry, it's important to be aware of this.

What exactly is agranulocytosis?

Simply put, Agranulocytosis is a life-threatening condition in which the number of white blood cells called neutrophils in our body drops to a very low level. Did you know that white blood cells are like soldiers in our immune system? They are the ones who protect us from disease-causing germs. When the number of these neutrophils decreases, our body becomes more susceptible to germs. Even germs that normally don't cause us much harm can make us sick. Imagine, if a special forces unit in our country's army suddenly disappeared, wouldn't even a small enemy be a big problem? This is the same. The name "agranulocytosis" comes from the word granulocytes . Granulocytes are also a type of white blood cell. They contain enzymes that destroy germs. There are three types of granulocytes: Neutrophils , Eosinophils, and Basophils . Neutrophils are the most abundant white blood cells in our bodies. That's why doctors test neutrophil levels to diagnose agranulocytosis. This is sometimes called granulocytopenia .

What is the difference between Neutropenia and Agranulocytosis?

Both are associated with a decrease in neutrophils. However, agranulocytosis is the most severe form of neutropenia .

• Neutropenia is a condition in which the number of neutrophils in the blood is lower than normal. People with fewer than 1,500 neutrophils per microliter (mL) of blood are said to have neutropenia.
• Agranulocytosis is the last, most dangerous stage, when the neutrophil count drops below 100 per microliter of blood. Got it? This is the most severe form of neutropenia.

Are there types of agranulocytosis?

Yes, this can come in two main ways: 1. Inherited agranulocytosis:This is caused by a genetic disorder. That is, there is a problem with the genes that help our body make neutrophils. This condition affects babies and young children the most. 2. Acquired agranulocytosis: This can have many causes. However, most of the time (about 70% of the time!) it is caused by a side effect of some medication we take. This type is most common in adults.

How common is this condition?

This is actually a very rare condition. On average, about seven people out of every hundred thousand develop this condition each year. So don't be unnecessarily scared, but it's important to be aware.

What are the symptoms of Agranulocytosis?

In this case, the main symptoms are related to infections. These can start suddenly, or they can increase gradually over time. See if these symptoms sound familiar to you:

• Getting a fever, feeling cold and shivering.
• Increased heart rate and breathing rate.
• Feeling of lifelessness and extreme fatigue.
• Sore throat (pharyngitis), gingival recession, bleeding gums.
• Sores in the mouth and throat, making it difficult to swallow food and drink.
• Sudden low blood pressure (hypotension), dizziness, fainting, and possibly even loss of consciousness.

If you have symptoms like these, especially if they occur frequently, you should definitely seek medical advice.

Why does agranulocytosis occur? What are the causes?

The main reason for this is a dangerous decrease in the number of white blood cells called neutrophils in our body. There are two possible reasons for this:

• Either our bodies don't make enough neutrophils to function properly.
• Otherwise, the neutrophils in the body are destroyed, or they die prematurely.

Now let's see what the specific reasons are for this.

Inherited agranulocytosis

In this, there is a problem with a gene that helps make neutrophils. This is a condition that is present at birth (congenital). For example, if we take Kostmann syndrome , or congenital agranulocytosis , a rare condition, newborn babies often have infections, fever, inflammation, and bone problems.

Acquired agranulocytosis

There could be a number of reasons for this. Let's see what they are:

• Certain medications: This is the most common cause . Some antibiotics , antipsychotic medications , and thyroid medications can cause this condition.
• Autoimmune diseases : These are diseases in which the immune system attacks the body's own cells. Examples include lupus and rheumatoid arthritis .
• Bone marrow diseases and bone marrow failure disorders: For example , aplastic anemia . The bone marrow is where our blood cells are made.
• Cancer: Cancers that affect the bone marrow, especially leukemia .
• Cancer treatments (Chemotherapy): These treatments can destroy healthy cells as well as neutrophils.
• Exposure to toxins or chemicals: Long-term exposure to things like lead or mercury.
• Infections: Severe infections like malaria , tuberculosis , and Rocky Mountain spotted fever .
• Nutritional deficiencies: Deficiency of essential nutrients like Vitamin B12 and Folate.

Who is most at risk for this condition?

Women are slightly more likely to develop this condition than men. Also, agranulocytosis can affect anyone of any age. However, the following people are at particular risk:

• People undergoing chemotherapy for cancer.
• People with autoimmune diseases like lupus and rheumatoid arthritis .
• People taking certain medications, such as Clozapine (Clozaril®) , an antipsychotic , Trimethoprim/sulfamethoxazole (Bactrim™) , and Methimazole (Tapazole®), a thyroid medication.

What are the complications that can occur due to agranulocytosis?

This condition can lead to frequent or long-term infections (chronic infections).

If agranulocytosis persists for more than three to four weeks, there is a 100% chance of developing an infection.

If left untreated, sepsis can lead to a severe, life-threatening immune system reaction called sepsis. Sepsis is a severe infection that spreads throughout the body. People over the age of 65 or those with other health problems, such as heart disease, kidney disease, or lung disease, are at higher risk of developing these complications.

How do you diagnose this?

How does a doctor diagnose agranulocytosis? They usually follow these steps:

• Complete Blood Count (CBC) and differential: This test looks at the different types of cells in your blood, especially the number of neutrophils. An absolute neutrophil count (ANC) of less than 100 per microliter of blood is considered agranulocytosis. (A normal value for a healthy person is greater than 1,500.)
• Bone marrow biopsy and aspirate: Sometimes, this test may be done to see how your bone marrow is producing neutrophils.
• Symptoms and medical history: The doctor will ask you about your symptoms, medications you take, recent infections, and any exposure to chemicals.
• Genetic testing: If a genetic condition is suspected, genetic testing may be done to confirm it. This may involve a skin biopsy or a blood test.

What are the treatments for this?

The good news is that there are treatments for this condition, agranulocytosis. Treatment depends on the cause.

• Stopping the Causative Medication: If a medication is causing your symptoms, the first thing to do is to safely stop taking it as directed by your doctor. Never stop taking medication without consulting a doctor.
• Antibiotics: If you have an infection, your doctor will prescribe appropriate antibiotics and other medications to reduce symptoms.
• Granulocyte colony-stimulating factor (G-CSF): This type of injection can be given to help the body produce neutrophils more quickly. Examples include drugs such as Filgrastim (Neupogen®) , Pegfilgrastim (Neulasta®) , and Lenograstim (Granocyte®) .
• Immunosuppressants: If the condition is caused by an autoimmune disorder, medications that suppress the immune system, such as Prednisone, may be prescribed.
• Bone marrow transplant: If other treatments are not successful, you may need to have a bone marrow transplant. In this, you will be given bone marrow from a healthy donor. This will help your body produce healthy neutrophils again.
• Preventing infections: This is very important. People with this condition need to be very careful to avoid infections.
• Always wash your hands thoroughly with soap.
• Avoid crowded places as much as possible.
• Wear a face covering/mask when going out.
• Talk to your doctor about what else you can do to prevent getting sick.

How quickly will I recover after treatment?

The recovery time depends on the cause of your low neutrophil count and the severity of the condition. If it's a side effect of a medication, it can usually take a week to three weeks for your neutrophil count to return to normal after you stop taking the medication.

Can this be prevented?

Sometimes, agranulocytosis cannot be completely prevented. However, if you are taking a medication that can lower your neutrophils (e.g., chemotherapy, some psychiatric medications), it is important to talk to your doctor and have your neutrophil levels checked regularly. If you are receiving chemotherapy for cancer, your doctor may recommend a G-CSF injection to help increase neutrophil production.

What is the outlook for someone with agranulocytosis?

Although this is a treatable condition, results can vary from person to person. With proper treatment, or if the medication that reduces neutrophils is stopped, the condition often resolves.

However, if left untreated, severe infections and even death can occur. The longer the neutrophil count remains at a dangerous level, the greater the risk of infection and complications. This is especially true for people over 65 and those with underlying medical conditions (such as kidney, heart, or lung disease).

When should you see a doctor?

Agranulocytosis is a life-threatening condition. Therefore, it is essential to get a diagnosis and treatment immediately. See a doctor immediately if you have any of these symptoms:

• If you get frequent infections.
• If the infection takes a long time to heal.
• If you develop symptoms of infection such as fever, chills, or sore throat.

If you have an autoimmune disease or are taking medication that lowers your neutrophils, don't skip your medical checkups. Regular checkups with your doctor can help you monitor your neutrophil levels and help prevent infections.

The most important things we need to remember (Take-Home Message)

Okay, so let's recap some of the most important points from what we've talked about:

• Agranulocytosis is a serious but treatable condition in which there is a severe decrease in white blood cells called neutrophils.
• If you have symptoms like frequent infections, fever, or sore throat, see a doctor immediately.
• This can be caused by certain medications, autoimmune diseases, and cancer treatments.
• Treatment may include stopping the offending drug, antibiotics, G-CSF injections, and possibly a bone marrow transplant.
• Protecting yourself from infections is very important. You can protect yourself by doing simple things like washing your hands frequently and wearing a mask when in public.
• If you are at risk for this (e.g., if you take certain medications), don't skip doctor appointments.

I hope this information is useful to you. Stay healthy!