What is Agranulocytosis?

Agranulocytosis is a potentially fatal illness characterised by abnormally low neutrophil counts. One component of your immune system is white blood cells. They keep your body safe from infection by scavenging pathogens.

Two Varieties of Agranulocytosis Exist

The inherited form of agranulocytosis is caused by a genetic disease that alters the production of neutrophils in the body. It is more prevalent in infants and young children.

Although acquired agranulocytosis can have a variety of reasons, up to 70% of instances are related to pharmaceutical side effects. The majority of cases of this type of agranulocytosis occur in adults.

Agranulocytosis Symptoms

Infection symptoms brought on by agranulocytosis might manifest quickly or gradually. Among the symptoms could be:

  • Fever and chills
  • Quicker respiration and heart rate
  • Weariness and weakness of the muscles
  • Sore gums that are inflamed and bleeding (pharyngitis)
  • Sores in your throat and mouth that interfere with swallowing
  • Unexpectedly low blood pressure, or hypotension, can make you feel weak or dizzy or even make you pass out.

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Why Does Agranulocytosis Occur?

A dangerously low neutrophil count results in agranulocytosis. This may occur when:

  • Your body doesn't produce enough functional neutrophils.
  • The neutrophils you do have are either dying off too soon or are being destroyed.

Risk Factors of Agranulocytosis

Agranulocytosis is somewhat more prevalent among women and those who are born with a female gender assignment.

  • It can strike anyone at any age.
  • An increased risk of agranulocytosis is seen in those who are undergoing cancer treatments, including chemotherapy.
  • Possess autoimmune disorders, such as lupus or rheumatoid arthritis, which induce your immune system to target a specific area of your body.

Consume certain prescription medications, such as;

  • Methimazole (Tapazole®)
  • Trimethoprim/sulfamethoxazole (BactrimTM)
  • Antipsychotic clozapine (Clozaril®)

What Agranulocytosis Complications Are There?

  • An individual with agranulocytosis is susceptible to recurrent or persistent infections.
  • When agranulocytosis persists for more than three or four weeks, infection occurs almost invariably.
  • If left untreated, agranulocytosis can result in sepsis, an overreaction of the immune system that can be fatal.
  • If you have additional medical issues such as lung, renal, or heart illness, or if you are over 65, you are at a higher risk of developing complications with agranulocytosis.
  • It is imperative that you cease using a prescription medicine if it is the cause of your symptoms.
  • Consult your healthcare provider about the proper way to discontinue.

Treatment for Agranulocytosis

  • Antibiotics: In order to treat your illness and reduce your symptoms, your doctor may recommend antibiotics in addition to other drugs.
  • Granulocyte colony-stimulating factor (G-CSF): In order to speed up your body's production of neutrophils, your doctor might advise injecting a medication. Lenograstim (Granocyte®), pegfilgrastim (Neulasta®), or filgrastim (Neupogen®) may be prescribed by your doctor.
  • Immunosuppressants: Your doctor may prescribe immunosuppressive medications, such as prednisone if agranulocytosis is brought on by an autoimmune disease.
  • Bone marrow transplant: You might require a bone marrow transplant if all other therapies fail. You acquire bone marrow from a donor during this process, so your body has more neutrophils to produce.

Agranulocytosis patients should take precautions to prevent infection. Avoid crowds, wash your hands frequently, and cover your face when you're out in public. Discuss more preventative measures you can take with your healthcare professional.

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Frequently Asked Questions

What causes agranulocytosis?

Agranulocytosis can be caused by various factors, including certain medications (e.g., chemotherapy, antithyroid drugs, antipsychotics, antibiotics), autoimmune disorders, bone marrow diseases, severe infections, and rare genetic disorders.

What is the prognosis for someone with agranulocytosis?

The prognosis differs based on the cause and promptness of treatment. Many patients recover fully with appropriate medical intervention, though some may experience recurrent or chronic issues.

Is agranulocytosis life-threatening?

Agranulocytosis can be life-threatening if not promptly treated, as it severely compromises the immune system, making the body vulnerable to severe infections.

How long does it take to recover from agranulocytosis?

Recovery time varies depending on the cause and treatment response. Some individuals may recover within weeks, while others may require prolonged treatment and monitoring.

Who is at risk of developing agranulocytosis?

Individuals taking certain high-risk medications, those with autoimmune disorders, bone marrow diseases, severe infections, or specific genetic predispositions are at higher risk of developing agranulocytosis. Check with your doctor before using these medicines.

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