Overview

Agranulocytosis is a potentially fatal blood disease. It occurs when the body does not produce enough neutrophils, a kind of white blood cell. White blood cells are immune system components. They defend the body from infection by combating microorganisms that cause illness.

Agranulocytosis, also known as granulocytopenia, is a severe type of neutropenia. Neutropenia is characterized by lower-than-normal amounts of white blood cells. People with low white blood cell counts are extremely susceptible to infection. They might become ill from bacteria that would not be hazardous normally. Agranulocytosis can be treated with drugs by healthcare experts. If left untreated, the disease might progress to serious illnesses. It can potentially cause sepsis (a severe infection response) and death.

Agranulocytosis can be hereditary or induced by autoimmune or bone marrow problems, infection, poor nutrition, cancer treatment (such as chemotherapy or bone marrow transplant), or exposure to poisons, chemicals, or drugs.

 

What are the Granulocytes & Agranulocytes?

Granulocytes and agranulocytes are types of white blood cells.

Granulocytes are a type of white blood cell that have granules, or small particles, within their cytoplasm. They include neutrophils, eosinophils, and basophils. These cells play a key role in the body's immune response and help to fight off bacterial and fungal infections.

Agranulocytes, on the other hand, are a type of white blood cell that do not have granules within their cytoplasm. They include lymphocytes and monocytes. These cells also play a key role in the body's immune response, but they are more involved in fighting viral infections, and some types of cancer.

Agranulocytosis is a condition characterized by a severe decrease in the number of granulocytes in the blood. This can make it difficult for the body to fight off infections and can be life-threatening.

 

What is the Function of Granulocytes?

Granulocytes collaborate to cleanse your body of illness or allergies. Neutrophils should account for 50% to 70% of all white blood cells in your body. Eosinophils should account for 1% to 3%, whereas basophils should contribute for 0.4% to 1%. Each granulocyte type has a unique mix of chemicals and enzymes in its granules. As a result, each category serves an unique function:

• Neutrophils: Neutrophils, the most frequent form of granulocyte, fight bacteria. In its lifespan, each neutrophil cell may eat up to 20 microorganisms. The most common kind of granulocyte is neutrophil. They account for around 40% to 60% of all granulocytes in your body. That amounts to almost two-thirds of all of your white blood cells.
• Eosinophils: Eosinophils are granulocytes found in nearly all immunological reactions, most notably allergies. They do, however, combat parasites.
• Basophils: Basophils are granulocytes that predominantly fight allergic responses. They produce histamine (which transports allergies out of your body) and heparin, a blood thinner (which prevents clotting).

 

What is Agranulocytosis?

Agranulocytosis is a condition characterized by a severe decrease in the number of white blood cells called granulocytes in the blood. This can make it difficult for the body to fight off infections and can be life-threatening. The condition can be caused by certain medications, infections, or underlying medical conditions. Symptoms of agranulocytosis include fever, sore throat, and mouth ulcers, as well as signs of infection such as redness, warmth, and swelling. Treatment typically involves stopping the use of any medications that may be causing the condition, and administering drugs to increase the number of white blood cells. In some cases, a person may need to be hospitalized for treatment.

 

How common is Agranulocytosis?

The exact epidemiology of agranulocytosis is not well-established. However, it is a relatively rare condition that is estimated to occur in 1 out of every 100,000 to 200,000 people.

Agranulocytosis can occur in people of all ages and genders, but it is more common in older adults and in people who are taking certain medications or have certain underlying medical conditions. The drugs most commonly associated with agranulocytosis are antithyroid drugs, antipsychotics, and non-steroidal anti-inflammatory drugs (NSAIDs).

There is also a higher incidence of agranulocytosis in people who have certain underlying medical conditions, such as cancer, autoimmune diseases, and genetic disorders.

It's also important to note that agranulocytosis can be caused by both environmental and genetic factors. Some studies have shown that people with certain genetic variations may be at an increased risk for developing agranulocytosis.

 

Types of Agranulocytosis

There are several different types of agranulocytosis, which are classified based on the underlying cause:

1. Drug-induced agranulocytosis: This type of agranulocytosis is caused by certain medications, such as antithyroid drugs, antipsychotics, and non-steroidal anti-inflammatory drugs (NSAIDs).
2. Infection-induced agranulocytosis: This type of agranulocytosis is caused by certain infections, such as streptococcal infections, viral infections, and bacterial infections.
3. Autoimmune agranulocytosis: This type of agranulocytosis is caused by an immune system disorder in which the body's immune system mistakenly attacks and destroys white blood cells.
4. Idiopathic agranulocytosis: This type of agranulocytosis is caused by an unknown or idiopathic (no specific cause) reason.
5. Neoplastic agranulocytosis: This type of agranulocytosis is caused by a cancer or tumor that produces granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF)
6. Genetic agranulocytosis: This type of agranulocytosis is caused by genetic defects that affect the production of white blood cells.

It is important to note that some cases of agranulocytosis may have multiple underlying causes, and a specific diagnosis can only be made by a health professional.

 

Risk Factors for Agranulocytosis

Agranulocytosis is a rare condition, but certain factors may increase the risk of developing it. These risk factors include:

1. Certain medications: Certain medications, such as antipsychotics, antibiotics, and anti-seizure medications, have been linked to an increased risk of agranulocytosis.
2. Autoimmune disorders: People with autoimmune disorders, such as lupus or rheumatoid arthritis, may be at a higher risk of developing agranulocytosis.
3. Cancer: Some types of cancer and cancer treatments, such as chemotherapy, can increase the risk of agranulocytosis.
4. Exposure to toxins: Exposure to certain toxins, such as pesticides or industrial chemicals, may increase the risk of agranulocytosis.
5. Genetic factors: Some genetic disorders, such as Kostmann syndrome, are associated with an increased risk of agranulocytosis.
6. Age: older people may have a higher risk of developing agranulocytosis.

It's essential to remember that having one or more of these risk factors doesn't guarantee you'll get agranulocytosis. However, if you have any of these risk factors and are experiencing agranulocytosis symptoms, you should seek medical assistance as soon as possible.

 

Symptoms & Signs of Agranulocytosis

Symptoms and signs of agranulocytosis can vary depending on the underlying cause and the severity of the condition, but they may include:

1. Fever: This is a common symptom of agranulocytosis and can be caused by an infection or inflammation.
2. Sore throat.
3. Mouth ulcers. 
4. Fatigue: This is a common symptom of agranulocytosis and can be caused by anemia or a lack of energy due to a lack of white blood cells.
5. Redness, warmth, and swelling.
6. Skin eruptions: This is a common symptom of agranulocytosis.
7. Shortness of breath.
8. Joint pain. 
9. Bruising or bleeding.
10. Frequent infections: This is a common symptom of agranulocytosis and can be caused by a lack of white blood cells.

It is important to note that some people with agranulocytosis may not have any symptoms and their condition is only discovered during a routine blood test.

 

How is Agranulocytosis Diagnosed?

Agranulocytosis is usually diagnosed through a combination of blood tests, physical examination, and medical history.

The primary test used to diagnose agranulocytosis is a complete blood count (CBC), which measures the number and types of white blood cells in the blood. Agranulocytosis is characterized by a severe decrease in the number of granulocytes in the blood.

Other tests that may be performed to help diagnose agranulocytosis include:

• Blood cultures to detect any underlying bacterial or fungal infections
• Bone marrow aspiration or biopsy to evaluate the cells that produce white blood cells
• A differential white blood cell count to determine the proportion of different types of white blood cells in the blood
• Additional tests to evaluate the cause of agranulocytosis, such as testing for autoimmune disorders or genetic conditions.

After the diagnosis, doctors will perform more tests to identify the cause and the underlying condition, such as toxicology tests, autoimmune tests, genetic tests, or others.

It is crucial to remember that only a skilled healthcare expert can make a diagnosis of agranulocytosis, and that a precise diagnosis can only be determined after evaluating a patient's whole medical history and symptoms.

 

How Agranulocytosis is Treated?

The treatment of agranulocytosis depends on the underlying cause of the condition.

1. If the agranulocytosis is caused by a medication, treatment typically involves stopping the use of the medication and switching to an alternative.
2. If the agranulocytosis is caused by an infection, treatment typically involves antibiotics, antivirals, or antifungals to treat the infection.
3. If the agranulocytosis is caused by an underlying medical condition, such as cancer or an autoimmune disorder, treatment typically involves addressing the underlying condition.
4. In all types of agranulocytosis, treatment may also include administering drugs to increase the number of white blood cells, such as granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF).
5. If the agranulocytosis is severe, hospitalization may be required to monitor the patient and provide supportive care, such as oxygen therapy, fluids, and antibiotics to prevent or treat infections.

It is important to note that the treatment of agranulocytosis is typically personalized to the individual and their specific condition, and it is important to follow the treatment plan prescribed by a healthcare professional.

Recovery from agranulocytosis can take several weeks to several months, depending on the underlying cause of the condition and the severity of the condition. In some cases, agranulocytosis may cause long-term complications or chronic conditions, which will require ongoing management and monitoring.

 

Complications of Agranulocytosis

Agranulocytosis is a serious condition that can lead to a number of complications, including:

1. Increased susceptibility to infections: The lack of granulocytes in the blood can make it difficult for the body to fight off infections, which can lead to serious and potentially life-threatening infections.
2. Sepsis: Agranulocytosis can lead to sepsis, a serious condition characterized by a widespread infection throughout the body.
3. Anemia: Agranulocytosis can lead to anemia, a condition in which there are not enough red blood cells in the blood to carry oxygen to the body's tissues.
4. Bleeding: Agranulocytosis can lead to bleeding, as the lack of platelets in the blood can make it difficult for blood to clot.
5. Tissue damage: Agranulocytosis can lead to tissue damage, as the lack of white blood cells can make it difficult for the body to fight off infections and inflammation.
6. Long-term complications: In some cases, agranulocytosis may cause long-term complications or chronic conditions, which will require ongoing management and monitoring.
7. Mortality: Agranulocytosis is a potentially life-threatening condition if it is not diagnosed and treated in a timely manner.

It is essential to remember that some persons with agranulocytosis may not exhibit any symptoms, and the issue is only detected via a regular blood test, which may aid in the prevention of the aforementioned consequences.

 

Agranulocytosis Precautions

If you have agranulocytosis, you are at high risk of catching any infection to which you are exposed. Infections are the chief complication of this disorder, and their occurrence depends on its duration and its severity. People whose neutrophil count stays below 100 per microliter for more than 3 or 4 weeks have an almost 100% incidence of infection.

Some precautions can help you stay safe, though:

• Eat only well-cooked food. Avoid salads, sauces, and undercooked or raw food like meat, shellfish, and eggs.
• Store and prepare food carefully.
• Avoid going to crowded places.
• Only eat fruits and vegetables that can be thoroughly washed or peeled.
• Avoid working with soil and animals.
• Wash hands frequently and maintain good personal hygiene.
• Use an electric shaver instead of razors.
• Don't swim in ponds and rivers and avoid shared hot tubs.
• Avoid close contact with sick people.

Agranulocytosis is a dangerous condition and can cause severe, life-threatening infections. Your doctor will try to find the cause, but many causes of agranulocytosis are reversible. 

Watch out for the signs of agranulocytosis if you know you have a condition associated with it. Your doctor will warn you before prescribing any medicines known to cause agranulocytosis. 

If you are affected by this disorder, treating infections early is vital to prevent them from escalating out of control. Alertness for signs of infection and quick action can save you from complications and life-threatening situations.

 

Outlook For Agranulocytosis

Agranulocytosis is a condition characterized by a severe reduction in the number of white blood cells called granulocytes. These cells play a crucial role in the body's immune system and are important for fighting off infections. The outlook for agranulocytosis can vary depending on the underlying cause and the severity of the condition. In some cases, agranulocytosis may be a temporary condition that can be treated and resolved with appropriate therapy. However, in other cases, it may be a more serious condition that requires hospitalization and intensive treatment. In rare cases, agranulocytosis can be fatal if left untreated. It's important to seek medical attention if you have symptoms of agranulocytosis and to follow your healthcare provider's recommendations for treatment.

 

Conclusion

Neutrophils are the most abundant white blood cell in blood and play a critical role in providing innate immunity against various offending agents. Agranulocytosis, also known as agranulosis or granulopenia, is an acute condition involving severe and dangerous neutropenia. Agranulocytosis is a condition in which the absolute neutrophil count (ANC) is less than 100 neutrophils per microlitre of blood. People with this condition are at a very high risk of severe infection. Broadly, it can be due to hereditary disease due to genetic mutation or acquired disease. Agranulocytosis can have various presentations, including fever, chills, sore throat, etc. It can be a life-threatening condition that requires prompt diagnosis and treatment.

Agranulocytosis is generally accompanied with a history of a new or changed drug, recent exposure to chemical or physical agents, or recent viral or bacterial infection. There may be a history of autoimmune illness or a significant family history of recurrent infection, which generally begins in childhood. Malaise, fever, and chills are common early symptoms, followed by infections in the form of ulcers, necrotizing lesions of the gingiva, floor of the mouth, buccal mucosa, throat, or other locations within the oral cavity. Another manifestation of the condition might be pharyngitis, which causes difficulties swallowing and many skin abscesses. Because agranulocytosis can manifest suddenly, sepsis may occur. A temperature of greater than 40 degrees Celsius, tachycardia, tachypnea, and hypotension are generally observed on examination.

Infection is the most common complication of agranulocytosis. The length and severity of agranulocytosis are closely related to the likelihood of infection. When the ANC persists below 100 cells per microlitre of blood for more than 3-4 weeks, the infection rate approaches 100%.

Another serious consequence of agranulocytosis is sepsis. Sepsis is a clinical illness caused by a dysregulated immune system response to infection. Because the number of mature granulocytes is drastically reduced in agranulocytosis, the body is unable to fight off the invading pathogens, resulting in sepsis, bacteremia, and septic shock. Septic shock is a kind of distributive or vasodilatory shock that causes circulatory and metabolic problems and has a higher fatality rate.