Agranulocytosis

What is Agranulocytosis?

Agranulocytosis is a condition marked by the inability of the bone marrow to produce enough or sufficient white blood cells leading to its decrease in count or the condition known as leukopenia. It is an acute and a rare condition that most commonly affects the neutrophil content of the white blood cells in which the decrease in count can lead to neutropenia.

Neutrophils are among the granulocytes that make up the white blood cells. It is classified according to its staining characteristic marked by neutral subtle granules. Neutrophils outnumbered all types of granulocytes found in the white blood cells and the absolute neutrophil counts are in relation to the production of the white blood cells. Neutrophils are the most abundant type of white blood cells and are generally an integral part of the immune system which are the first to respond during and inflammatory process and during an infection from harmful microorganisms.

Agranulocytosis is a severe deficiency in the production of white blood cells that the onset can greatly affect the immune system of an individual. People with this deficiency are potentially at risk for infection as a consequence of suppressed immune system. The minor infection can become serious in individuals suffering from agranulocytosis while harmless microbes can potentially become strong enough to cause harm and attack the own body of the immune system.

The decrease in the production of white blood cells is the defining characteristic of agranulocytosis. The decrease in the white blood cell of an individual puts one at greater risk for infection as the function of which is to defend the body from harmful effects of microbes or other microorganisms. It is an essential component of the immune system that act by identifying harmful microbes, destroys them and remove the pathogens including the damaged cells and other harmful foreign materials in the body.

Symptoms of Agranulocytosis

The onset of agranulocytosis can be asymptomatic but if clinically present the symptoms may include the following:

• Sudden onset of high fever and chills.
• Incidence of sore throat.
• Weakness of the limbs may also be experienced.

People with agranulocytosis and suffering from an infection in any part or organ of the body will have a rapid progression of infection. Agranulocytosis can also hasten the progression of a life-threatening medical condition known as sepsis.

The main affected in the process of agranulocytosis is the production of a type of white blood cells called neutrophils. The alteration in the white blood cell count affecting the neutrophils can lead to neutropenia where the number of neutrphils in the blood stream is greatly diminished thereby resulting to symptoms such as:

• Gum disease that includes gum bleeding, mouth ulcers, halitosis and an increase in the production of saliva.
• Bacterial pneumonia
• Urinary tract infection.
• Gastrointestinal tract infection.
• Fungal infections

The infection in neutropenia may be localized to a certain area of the body but it can also spread via the bloodstream which in turn can spread to other areas and organs of the body.

Agranulocytosis Causes

The cause of agranulocytosis is classified into two forms namely:

Congenital agranulocytosis which is a form of agranulocytosis that is present at birth and is implicated on genetic imperfection.

Acquired agranulocytosis is a form of agranulocytosis as a consequence of medication and other form of treatment which can affect the bone marrow and its production of the blood cells. Acquired agranulocytosis may be due to the following treatments:

• Anticonvulsant drugs that are used for treating or managing epileptic seizure and this drug may include barbiturates, aldehyde, aromatic allylic alcohol, benzodiazepines and other anticonvulsant drugs.
• Antithyroid drugs such as methimazole, carbimazole and propylthiouracil.
• Antibiotics such as cotrimoxazole and penicillin.
• Nonsteroidal anti-inflammatory drugs such as metimazole and naproxen.
• Mirtazapine is an anti-depressant drug considered in agranulocytosis due to its side effect of bone marrow suppression.
• Antipsychotic drugs such as clozapine.

Other causes of acquired agranulocytosis include the following:

• Cocaine abuse
• Chemotherapy treatment for cancer.
• Diseases involving the bone marrow such as aplastic anemia.
• Cancers or tumor growth involving the bone marrow such as leukemia.
• Chemical toxins and radiation exposure.
• Vitamin B-12 and folate deficiency.
• Diseases involving the autoimmune such as systemic lupus erythematosus.
• Spleen enlargement
• Autoimmune diseases

Agranulocytosis Treatment

Agranulocytosis often responds well to treatment. The prognosis is positive for those diagnosed and treated promptly while it is potentially life-threatening if left untreated. Mortality in agranulocytosis is associated with infection due to the rapid progression and multiple organ involvement.

Close monitoring of serial blood counts is necessary in agranulocytosis that is asymptomatic. Withdrawal from medications and other offending agent affecting the bone marrow and the white blood cell production is also necessary but upon the advice of a doctor.

Antibiotic and antifungal treatment is usually given for agranulocytosis that resulted from an infection from bacteria and fungus and other form of infection.

Colony-stimulating factor encourages more production of neutrophils in the bone marrow and is beneficial for agranulocytosis patient undergoing chemotherapy treatment.

Leukocyte transfusion may also be beneficial for agranulocytosis patients to replace the deficit in the granulocytes especially the neutrophils