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What is Agranulocytosis Disorder?

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  • Written By: Mary McMahon
  • Edited By: O. Wallace
  • Last Modified Date: 29 August 2016
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Agranulocytosis disorder is a form of leukopenia, or shortage of white blood cells, which involves critically low levels of granulocytes. Granulocytes are specialized white blood cells which support the immune system with a variety of compounds they carry inside their bodies in tiny granules which can burst open as required. This condition may also sometimes be referred to as neutropenia, referencing a specific type of granulocyte found in the blood. A patient with agranulocytosis disorder can face critical health problems, and in some cases, the condition is fatal.

There are two primary reasons for there to be a shortage of granulocytes in the blood. The first is lack of production in the bone marrow, and the second is destruction at a rate which is too high for the bone marrow to make replacements. Sometimes, people develop agranulocytosis disorder spontaneously, but more commonly it is linked with the use of medications and treatments such as sulfonamides, antithyroid drugs, chemotherapy, phenothiazines, or radiation.

Patients may display no symptoms at first, but they commonly experience acute infections as a result of the lack of granulocytes to fight infection. Lesions commonly appear in the mouth and along other mucus membranes, and upper respiratory infections are very common. The patient may also feel fatigued or run down, and commonly a high fever develops as the body struggles to fight even the smallest infection.

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Doctors can diagnose agranulocytosis disorder with the use of a blood count, in which the levels of white blood cells and granulocytes in particular can be determined. Once a diagnosis is made, the doctor must determine the cause of the condition, as it cannot be resolved without dealing the underlying cause. In the case of agranulocytosis disorder caused by medication, treatment usually starts with withdrawing the medication.

The patient is also extremely vulnerable to infection, so isolation is usually recommended to minimize contact with people who could be carrying infections. Antibiotics and aggressive treatment are also used at the slightest signs of infection, to defend the body while it rebuilds its white blood cells. In some cases, a bone marrow transplant may be used so that the patient can start producing more white blood cells, including granulocytes.

Even with the best treatment, agranulocytosis disorder can be deadly for the patient. It may not be possible to address the cause of the condition in time, for example, especially if the cause is not apparent, and even a mild infection can overwhelm antibiotics and kill a patient who lacks granulocytes.

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