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Agranulocytosis – What Happens When The Defense Goes Down

When the bone marrow is not able to provide adequate amount of neutrophils, a type of white blood cell that responds primarily when there is infection. This condition is called agranulocytosis, which roughly translates to absence of formation of granulocytes (white blood cells). Other names include granulocytopenia and granulopenia. When agranulocytosis is present, the body’s defenses against diseases falls down alarmingly that infection precaution is immediately instituted. This can potentiate less serious infections and may promote invasion of opportunistic microorganisms in the body.

agranulocytosis-low-white-blood-cells

image credit:hospitalsoup.com

Although it is a rare condition, agranulocytosis can be precipitated by various risk factors. Women are more likely to develop agranulocytosis than men. Inherited agranulocytosis is found in children while acquired form of agranulocytosis is common in elderly.

Agranulocytosis may be acquired or congenital. Acquired agranulocytosis is usually caused by drugs and chemotherapy. Its onset can either be gradual or abrupt. Other causes of acquired agranulocytosis include clozapine and antithyroid medications, exposure to radiation, toxins, and chemicals, low Vitamin B12 (Cobalamine), B9 (Folate), and protein, infections, bone marrow transplants, and blood transfusions. On the other hand, congenital agranulocytosis is present at birth. It has two subtypes: cyclic neutropenia (21-day period of up and down cycle) and severe congenital neutropenia. Congenital agranulocytosis is caused by a genetic defect.

Generally, early symptoms of agranulocytosis include fever, chills, easy fatigability, sore throat, mouth sores, yellowish discoloration of the skin and bleeding gums. Children with congenital agranulocytosis may be observed as having poor weight gain. Other symptoms include fast breathing and heart rate, and skin infections.

Diagnosis of agranulocytosis involves a thorough assessment of medical history: drugs being taken, recent infections, and history of autoimmune diseases. White blood cell counts are done using both blood and urine samples. If bone marrow affectation is suspected, bone marrow test is done. Also, if autoimmune diseases are suspected, tests are done to validate them. Possible genetics counselling is ordered for congenital form of agranulocytosis.

Treatment depends on the type of agranulocytosis and other factors involved. The best treatment is transfusion of neutrophils and/or white blood cell stimulating factor. Neutrophils replace the lost white blood cells while white blood cell stimulating factor stimulates the body to produce adequate amount of white blood cells. Antibiotics, antivirals, and antifungal medications are given to counteract persisting infections. Also, if the patient is taking several drugs, the doctor may advise him to stop taking these drugs to identify which causes the problem. Alternative drugs are then given. Removal of toxins that cause agranulocytosis may be done too.

Once identified early, prognosis is good. If undergoing chemotherapy and the drugs needed to be taken can cause agranulocytosis, doctors order series of blood tests to monitor the levels of white blood cells from time to time. Through this, doctors may know when to temporarily stop therapies and to give medications that could stimulate production of white blood cells. If at risk for developing agranulocytosis, patients are advised also to increase intake of green leafy vegetables and fresh fruits particularly, citrus fruits. However,once agranulocytosis are present, fresh fruits and vegetables are avoided since they may harbour disease-causing microorganisms.

Without treatment, patient can die because of infections.

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