Clinical picture and causes of agranulocytosis
Agranulocytosis is a disorder in the formation of granulocytes , a group of white blood cells ( leukocytes ) . The clinical picture of the disease is fulfilled when the number of granulocytes in the blood falls below 500 cells per milliliter.
Formation disorder of granulocytes
Due to the extreme reduction in granulocytes, the immune system collapses. The body can no longer defend itself against viral and bacterial infections, as well as against mycoses.
In the worst case, agranulocytosis can lead to sepsis. The disease is triggered by antipyretics, analgesics, sulfonamides or antithyroid drugs. In rare cases, tumors or aplastic anemia are the cause.
The rare and life-threatening side effect agranulocytosis
Drugs often cause side effects in the body, which are mostly harmless and short-lived. However, some can trigger a potentially fatal condition called agranulocytosis.
In the term agranulocytosis, the “A” at the beginning means “no”. This results in the translation “no granulocytes”, but a significant reduction is already sufficient for the disease. If the white blood cells in the blood are reduced by a certain value, the disease overrides the immune system. Viruses and bacteria find their way into the body.
The details of drug-induced agranulocytosis are not fully understood. Doctors assume two pathogenetic causes:
- an allergic-mediated reaction
- or a toxic-mediated reaction.
Agranulocytosis as a toxic reaction develops slowly because there are still functioning defense cells in the body. It depends on the dose and how long you take the drug.
The immune reaction to already circulating granulocytes is an allergic reaction, which the body destroys in the short term. It occurs acutely and is neither dependent on the dose nor on the duration of ingestion. Whether the drug enters the body orally or intravenously probably does not affect the course.
The main characteristics of the “health guard” leukocytes
The leukocytes (white blood cells) are formed in the bone marrow, from there they migrate into the bloodstream, where they play an important role in the immune system. They protect the body from infections, fight inflammation, tumor cells and toxins. They are divided into three subgroups, of which only one group is affected by agranulocytosis. Lymphocytes form the body’s specific defense system. Monocytes migrate from the bone marrow into the tissues, where they mature into macrophages , the so-called scavenger cells.
The granulocytes are in turn divided into three groups. The basophilic granulocytes help to heal inflammation. Neutrophilic granulocytes absorb microorganisms and destroy them with enzymes. Eosinophilic granulocytes play an important role in the defense against parasites and the control of allergic reactions.
If the granulocytes, which take over the most active part of the immune system, are missing due to agranulocytosis, this has fatal effects on health.
The typical nonspecific course of the disease agranulocytosis
In the case of the typical course, experts also speak of the Triassic:
- Fever,
- Angina tonsillaris,
- Stomatitis aphthosa.
The first signs are non-specific flu-like symptoms. Above all, fever, chills, headache and joint pain, loss of appetite and nausea, as well as a general feeling of being unwell are typical. In the further course, inflammations in the throat, mouth, nose, anal or genital area occur due to the weakened immune system. The angina tonsillaris is typical – an acute inflammation of the tonsils. Multiple skin and mucosal necrosis and pneumonia can also occur. Aphthous stomatitis is an inflammation of the oral mucosa, which causes whitish defects in the mucosa.
If agranulocytosis is not recognized and treated in time, the symptoms lead to sepsis in 60 percent of cases. Patients who are elderly or debilitated by illness are at high risk of death.
Establishing the diagnosis and the type of therapy
Since the body is prone to infections, especially of the mucous membranes, due to agranulocytosis, it is difficult to recognize the disease. The symptoms are usually similar to a protracted flu infection, which the doctor tries to treat with common medication. Anyone taking medication that promotes agranulocytosis should be wary of flu-like symptoms. Early detection of blood changes can ensure survival.
Agranulocytosis is diagnosed with a blood test. The leukocytes are measured. If their number is below the above value, the patient is probably suffering from the rare side effect. If he is not taking any of the risky drugs, a biopsy of the bone marrow will provide information about the cause.
The next step is to stop taking medications that promote agranulocytosis. The number of leukocytes then increases within a few days. Until the normal value is reached, the patient takes drugs that temporarily replace the action of the immune system.
Agranulocytosis, which is usually triggered by medication, completely disables the immune system. If a doctor does not recognize and treat the disease in time, it can be fatal. The chances of a cure with early detection are high.
