Agranulocytosis
Agranulocytosis is a life-threatening blood disorder that occurs when the body does not produce enough of a type of white blood cell, or neutrophils.
White blood cells are part of the immune system. They protect the body from infection by fighting disease-causing germs.
You need to know, agranulocytosis is also a severe form of neutropenia. Neutropenia is a lower-than-normal level of white blood cells. People with this condition are very susceptible to infections.
If the infection is not treated, this condition can cause severe infection, even sepsis and death.
Symptoms of Agranulocytosis
Agranulocytosis can sometimes have no signs or symptoms when there is no infection. Early signs and symptoms of agranulocytosis can include:
- A sudden onset of fever.
- Shivering.
- Sore throat.
- Fatigue.
- Joint and muscle pain.
- Pain in the mouth and gums.
- Ulcer.
- Bleeding gums.
Other signs and symptoms of agranulocytosis may include:
- Increased heart rate.
- Rapid breathing rate.
- Decreased blood pressure.
- Abscess on the skin.
Risk Factors for Agranulocytosis
Women are more likely to develop agranulocytosis than men. It can occur at any age.
However, inherited forms of the condition are more common in children, who usually die from the condition before reaching adulthood. Acquired agranulocytosis is most common in older adults.
Causes of Agranulocytosis
There are two types of agranulocytosis with different causes, namely inherited agranulocytosis and acquired agranulocytosis.
- Inherited agranulocytosis is caused by a genetic disorder. It affects the way the body produces white blood cells. Meanwhile, infantile genetic agranulocytosis is a rare form of congenital agranulocytosis. It is also known as Kostmann syndrome or congenital agranulocytosis. Newborns with this condition experience recurrent infections, fever, inflammation, and bone problems.
- Acquired agranulocytosis can occur as a result of certain infections. Exposure to chemicals and some prescription drugs are also causes. Drugs used to treat cancer and antipsychotics commonly cause acquired agranulocytosis. This form of the disorder is also called drug-induced agranulocytosis.
A study showed that as many as 70 percent of agranulocytosis cases were related to the use of antipsychotic drugs (for example, clozapine), antimalarials, anti-inflammatories, and antithyroid drugs (such as carbimazole).
Causes of acquired agranulocytosis include:
- Autoimmune conditions, such as lupus and rheumatoid arthritis.
- Diseases that attack the bone marrow, such as aplastic anemia, leukemia, and myelodysplastic syndrome.
- Hepatitis.
- HIV.
- Chemotherapy and bone marrow transplantation.
- Exposure to chemical compounds, such as arsenic or mercury.
- Infectious diseases, such as malaria, tuberculosis, and Rocky Mountain spotted fever.
- Lack of vitamin B12 and folic acid.
- Medications, such as antipsychotic drugs, malaria drugs, NSAIDs, and hyperthyroid drugs.
Diagnosis of Agranulocytosis
The doctor will check the patient’s medical history and perform a physical examination. If someone suspects symptoms of agranulocytosis, there are several tests that need to be done, such as:
- Complete blood count. This is done to determine the number of blood cells in the body, including white blood cells. The results of the blood test are used to determine further diagnostic measures.
- An absolute neutrophil count is also performed to complement a complete blood count.
- Bone marrow aspiration and bone marrow biopsy. This is done by taking blood and tissue samples from the bones of the pelvic area. Bone marrow is one of the places where blood cells are produced.
- Genetic testing. If a genetic disease is suspected, the doctor will ask for a genetic test.
Agranulocytosis Treatment
Treatment for agranulocytosis will be adjusted to the cause. However, there are several treatments that are usually done to treat agranulocytosis, such as:
- Use of antibiotics. If you previously had a history of infectious diseases, usually the doctor will provide treatment in the form of antibiotics.
- Granulocyte Colony Stimulating Factor (G-CSF) injection. This medication can be used to help the body produce white blood cells faster, especially neutrophils.
- Immunosuppressants. By giving this type of drug, the drug functions to suppress the body’s excessive immune response.
- Bone marrow transplant. If all treatments are not working optimally, the last treatment is a bone marrow transplant.
- Granulocyte infusion. In rare cases, a person may undergo a granulocyte transfusion, which is similar to a blood transfusion. Donor cells must be taken, ideally from a close relative. Steroids and G-CSF are given to the donor to increase their granulocyte production. After the blood is removed, the granulocytes are separated for transfusion into the recipient.
Prevention of Agranulocytosis
One way to prevent agranulocytosis is to avoid taking medications that can trigger this disease.
Have regular blood tests to check your neutrophil levels if you are taking long-term medications that can trigger agranulocytosis.
The doctor may recommend stopping medication intake if there is a decrease in the neutrophil count in the blood.
In addition, people with agranulocytosis must also take the following preventive measures:
- Avoid crowds.
- Avoid consuming fruits and vegetables that you cannot peel yourself or that are not clean.
- Avoid doing activities on the ground or with animals.
- Never eat undercooked or raw food.
- Maintain good personal hygiene.
- Wash your hands regularly with soap and water.
- Store and prepare food properly.
Complications of Agranulocytosis
Because agranulocytosis makes sufferers susceptible to infection, it can be very dangerous if not treated promptly. One complication of agranulocytosis is sepsis, a form of blood infection. Without treatment, sepsis can be fatal, even life-threatening.