Agranulocytosis is a life-threatening blood disorder. It happens when the body doesn’t make enough of a type of white blood cells called neutrophils. White blood cells are part of the immune system. They protect the body from infection by fighting off germs that make us sick.

Also known as granulocytopenia, agranulocytosis is a severe form of neutropenia. Neutropenia is lower-than-normal levels of white blood cells. People with a low white blood cell count are very vulnerable to infection. They may get sick from germs that wouldn’t be harmful otherwise. Healthcare providers can treat agranulocytosis with medications. Untreated, the condition can lead to severe infections. It can even lead to sepsis (a severe reaction to infection) and death.

Types

There are two types of agranulocytosis. It can be inherited (present at birth) or acquired.

Inherited agranulocytosis results from a genetic disorder. This problem affects how the body produces white blood cells. Infantile genetic agranulocytosis is a rare form of inherited agranulocytosis. It is also called Kostmann syndrome or congenital agranulocytosis. Newborns with this condition have recurring infections, fevers, inflammation and problems with their bones.

Acquired agranulocytosis can result from certain infections. Exposure to chemicals and some prescription medications may also cause it. Cancer-treating drugs and antipsychotic medications commonly cause acquired agranulocytosis. This form of the disorder is also called drug-induced agranulocytosis.

Causes

Several types of prescription medications cause acquired agranulocytosis. Infections, other health conditions and exposure to chemicals can also lead to the disorder. Causes of agranulocytosis include:

• Autoimmune disorders, including lupus and rheumatoid arthritis.
• Bone marrow disease and bone marrow failure disorders like aplastic anemia.
• Chemotherapy.
• Exposure to toxins or chemicals, such as lead or mercury.
• Infections such as malaria, tuberculosis and Rocky Mountain spotted fever.
• Prescription drugs, including some antibiotics, antipsychotic medications and drugs to treat thyroid problems.
• Poor nutrition and vitamin deficiencies.

Symptoms

Agranulocytosis itself doesn’t have symptoms. People with the disorder have symptoms of infection. Signs of agranulocytosis may appear suddenly or gradually. They include:

• Fever and chills.
• Faster heart rate and breathing.
• Sudden low blood pressure (hypotension), which can make you feel lightheaded or weak.
• Muscle weakness and fatigue.
• Sore mouth and throat and bleeding, inflamed gums.
• Ulcers (sores) in the mouth and throat that make it difficult to swallow.

Diagnosis

Neutrophils are a type of white blood cell that fights infections in the body. Agranulocytosis occurs when the absolute neutrophil count (ANC) is less than 100 neutrophils per microliter of blood. Usually, people have at least 1,500 neutrophils per microliter.

To diagnose agranulocytosis, your provider will order a blood test. This test, called a complete blood count (CBC), checks neutrophil levels in your blood. Your healthcare provider may also take a sample of your bone marrow to evaluate how your body produces white blood cells. Your provider will also ask about your symptoms, medications, recent infections and chemical exposure. If your provider believes you have the inherited form of the disease, you may need a genetic test.

Treatment

If a prescription medication is causing your symptoms, it’s essential to stop taking the medicine. Talk to your provider about how to stop safely. Agranulocytosis treatment includes:

• Antibiotics
• Granulocyte colony-stimulating factor (G-CSF)
• Immunosuppressant drugs
• Bone marrow transplant
• Preventing infection

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