Agranulocytosis – causes, symptoms & therapy

Agranulozytose

Agranulocytosis refers to a severe reduction in granulocytes, a subtype of white blood cells, to less than 500 cells per microliter of blood. It can occur as a side effect of various medications, as an expression of severe tumor diseases in the bone marrow, an aplastic anemia or as an accompanying phenomenon of chemotherapy.

What is agranulocytosis?

Drugs from completely different classes of action can sometimes show the same typical side effect. In the case of agranulocytosis, taking the painkiller novalgin, the neuroleptic clozapine or the popular antibiotic cotrimoxazole can lead to a disorder in the formation of white blood cells, which in the worst case can temporarily disable the body’s immune system. Agranulocytosis can also occur in severe cancers.

The word “A-granulocytosis” actually means “no granulocytes”, but a drop below 500 per microliter in the blood is enough to define agranulocytosis. Granulocytes are a subdivision of leukocytes , i.e. white blood cells, and there they mainly cover the work area “defense against bacterial pathogens , fungi and parasites “. They are everywhere on mucous membranes, in tissues and in the blood, where the body has to constantly deal with new and well-known pathogens so that we don’t get sick.

Like most blood cells, these granulocytes are formed in the bone marrow, which takes a few days. Due to the constant consumption of defense cells, new granulocytes are constantly being supplied from the bone marrow.

causes

In order to understand the exact pathogenesis of agranulocytosis, it has to be divided into two forms: The first form is an immune reaction against already circulating granulocytes, which are destroyed in the short term; the second form is triggered by toxic damage to the bone marrow and appears somewhat more slowly and leisurely, since functional defense cells are initially still on the move in the body.The first form is therefore a kind of allergic reaction of the body to medicines – metamizole or clozapine make a side effect in this way in people who are prone to such allergies .

In the second form, the drug causes damage directly or there are other causes of damage to the bone marrow – the antithyroid drug thiamazole inhibits an important enzyme in the blood formation of white blood cells in this way and prevents further maturation of the granulocytes with the result that the supply is stopped the bone marrow.

Co-trimoxazole can also have a direct toxic effect on the bone marrow, which corresponds to its main effect. Anyone can be affected by this from a certain dose, regardless of whether they are prone to allergies or not. Leukemia can also spread in the bone marrow and suppress normal blood formation, so that agranulocytosis can be triggered in this way. Since rapidly dividing cells are indiscriminately attacked and destroyed during chemotherapy, the bone marrow and the formation of red and white blood cells are also one of the first to suffer.

symptoms and course

Possible signs of agranulocytosis:

The problem with agranulocytosis is that it temporarily disables the immune system. Accordingly, one tends to infections, especially of the mucous membranes. The disease usually begins acutely (in the case of the first form) or within a few days (second form) with a disturbed general condition and fever. The mucosal infections usually appear in the form of a blistering viral infection of the oral mucosa ( aphthous stomatitis , especially herpes viruses ) or tonsillitis (angina tonsillaris) with enlargement and reddening of the pharyngeal tonsils, often with whitish coatings.

Mucosal ulcers in the entire gastrointestinal tract or necrosis of the outer skin can also form. Fungal infection of the mucous membranes appears as a crumbly, whitish coating. Pneumonia , diarrhea and other infections throughout the body also occur more easily because the pathogens are no longer contained by the granulocytes of the immune system.

Diagnose

The diagnosis of agranulocytosis is very simple: the granulocytes can be measured directly in the blood (differential blood count ). Together with the history of suspicious drug use, the case is usually clear. If there is a suspicion of a blood formation disorder of another cause, a bone marrow biopsy can be taken, which is usually done from the iliac crest.

complications

If agranulocytosis is not treated properly, it can lead to patient death due to various infections. As a rule, the patient suffers from a general malaise and feels weak. He is no longer able to carry out certain activities and often suffers from fever. There are also severe headaches and general fatigue.

Pneumonia can also occur if the respiratory tract becomes infected . Diarrhea and vomiting occur with infections of the stomach. The patient’s quality of life is greatly reduced by the agranulocytosis.

Tonsillitis can also occur. A diagnosis cannot be made quickly in every case, since the agranulocytosis can only show up a few weeks after taking the drug. Furthermore, a toxic reaction can occur, which is life-threatening and must be treated in any case. The treatment is carried out with the help of antibiotics and increased hygiene to prevent further infections or inflammation. The medication is also discontinued. Other complaints are treated symptomatically, with no further complications occurring in most cases. Life-threatening situations can only arise if severe symptoms occur if agranulocytosis is left untreated.

treatment and therapy

To treat agranulocytosis, the drug that caused it must first be stopped to prevent further damage. The blood formation then recovers by itself within a few days and the number of granulocytes increases to the extent that the bone marrow can supply them again. To bridge the time, prophylaxis with antibiotics and antimycotics , i.e. agents against bacteria and fungi, makes sense. This then practically replaces the body’s own defense system for a few days. In severe cases, granulocyte reproduction can also be accelerated with the growth factor G-CSF (granulocyte colony stimulating factor).

prevention

In order to prevent the named side effects, it is first important to limit the intake of the suspicious drugs to those cases that are really necessary and then to monitor them carefully in order to notice a drop in the granulocyte concentration in the blood in good time.This applies in particular to medications that are often administered, such as metamizol (Novalgin pain reliever) or the neuroleptic clozapine. Other drugs include clomipramine, sulfasalazine, ticlopidine, the antithyroid drugs thiamazole and carbimazole, and the antibiotic cotrimoxazole. Patients prone to allergic agranulocytosis should not receive these drugs.

If agranulocytosis is present, careful attention should be paid to mouth, throat and anal hygiene, and crowds should be avoided due to the risk of infection.

Dorothy Farrar

Hello and welcome to my Health Guide & Encyclopedia! My name is Dorothy Farrar, and I'm the founder and main author of this platform.
My passion for health and wellness started at a young age when I became interested in the connection between the food we eat and the way we feel. This fascination led me to study nutrition and dietetics in college, where I learned about the importance of a balanced diet and the impact of various nutrients on the body.

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