Agranulocytosis – life-threatening and widely underestimated
The lack of granulocytes , one of the three types of white blood cells, causes agranulocytosis. In Europe, between 1.6 and 9.2 people per million people contract the disease each year. The causes are unrecognized risks of certain medications and poor monitoring of blood values. If the disease breaks out, there is a risk of death, and therapy is reliably possible if used in good time.
There are three groups of white blood cells in human blood. These three groups show different forms of appearance under the microscope. In addition to monocytes and lymphocytes, granulocytes in different variants are involved in the human immune system.
Decreased immune defense in agranulocytosis
Granulocytes, in turn, occur in three subgroups. As neutrophilic, eosinophilic and basophilic granulocytes, they destroy micro-organisms and parasites. They also influence the body’s inflammatory response to invaders.
If the number of granulocytes falls below 3,000 cells per microliter of blood, the doctor speaks of granulocytopenia . The most severe form of this disease is called agranulocytosis. With this finding, the number of white blood cells is below 500 cells per microliter of blood. A healthy person has between 4,000 and 10,000 of these cells per microliter of blood.
Two main causes of agranulocytosis
The most common cause of agranulocytosis is intolerance to certain drugs. These include
- Thyreostatika wie Thiamazol, Carbimazol,
- Antibiotika wie Penicillin, Metronidazol,
- Antipyretika wie Phenylbutazon,
- Analgesics such as Metamizole, Paracetamol, Diclofenac, Ibuprofen,
- neuroleptics,
- Antiarrhythmika,
- antiepileptic drugs,
- Kardiaka,
- Gastrointestinal drugs such as cimetidine, metoclopramide
- Psychotropic drugs such as clozapine, fluoxetine
When the body responds to one of these drugs, allergic agranulocytosis occurs. The doctor calls this variant type 1 agranulocytosis. It occurs as an acute illness.
Anyone who takes cytostatics also runs the risk of developing agranulocytosis. In this case, there is no allergic reaction. The disease arises from the proliferation-inhibiting effect of these drugs. That is, they prevent the growth of new granulocytes. A third less common cause of agranulocytosis is osteomyelofibrosis. This is a blood formation disorder in the bone marrow. Type 2 agranulocytosis is present in the latter two cases. She is gradually making herself felt.
Two special cases are infantile and cyclic agranulocytosis. Experts call the former Kostmann syndrome. This is a hereditary disease. The number of neutrophilic granulocytes is too low. In the cyclic form of the disease, there is a lack of white blood cells at intervals.
Symptoms and clinical picture of agranulocytosis
The lack of white blood cells initially causes a poor general condition and causes fever. Patients feel flu-like symptoms and suffer from chills. This is followed by swollen mucous membranes and necrosis in the skin area. Sometimes lymphomas occur.
The disease often progresses in three stages:
- Fever,
- tonsillitis,
- mouth rot.
Inflammations in the genital or anal area and pneumonia are also possible. 60 percent of patients develop blood poisoning without treatment. These symptoms of the disease require thorough personal hygiene in order to prevent further diseases and infections.
Identify and treat agranulocytosis
The disease is diagnosed by the doctor simply and reliably with a differentiated blood count . Apart from that, he compiles a list of all risk drugs that trigger the allergic form of agranulocytosis. In order to rule out the presence of a non-drug-induced variant, he may take a tissue sample from the bone marrow.
In the case of the allergic form, the decisive therapeutic measure is to discontinue risk medication. The doctor replaces these with proven non-harmful active ingredients. In addition, he treats the disease with broad-spectrum antibiotics. They fight the feverish and inflammatory side effects of agranulocytosis. As a result, about five out of every hundred people who get sick die in Europe. This means a decline of around 15 percent compared to the end of the last century.
The time factor plays a major role
In the case of the allergy-related variant of the disease, the consequences are quickly noticeable in many cases. A few doses of a risk drug are enough to cause severe symptoms of the disease. In this case, the therapeutic measures come too late and deaths due to multiple organ failure cannot be ruled out. Type two progresses more slowly and the chance of a timely correct diagnosis is greater.
Risk factors for developing agranulocytosis
Despite improved observation methods, between one and nine people per million inhabitants still fall ill in Europe every year. Clearly identified risk factors for allergy-related type 1 are old age and taking drugs classified as high-risk. The extent to which gender and race influence the risk of contracting the disease is the subject of intense debate.
Risk drugs continue to be a problem
One reason for the constant number of new cases is the ignorance of many doctors regarding the side effects of certain drugs. In addition, there is insufficient observation of patients and a late diagnosis of the disease.
In many cases, the legal requirements do not correspond to the recognized risks of certain medicinal products. There are now extensive lists of drugs that trigger agranulocytosis. When they are administered, close monitoring of leukocytes is indicated. This is required by law in rare cases.
Observations on the diseases and their course show that there is an increased risk with so-called co-medication. This means that taking several high-risk drugs together increases the chance of developing agranulocytosis.
The exact mechanisms of action are often in the dark
Although the drugs mentioned above and many others have been identified as possible causes, the underlying mechanisms remain unclear. A further complication for basic research is that no reliable results can be obtained from other medications administered in parallel.
Agranulocytosis remains dangerous but manageable
Anyone who takes risk medication with regard to agranulocytosis is well advised to have regular blood count checks. This is followed by careful self-observation. As long as there are no adequate legal measures, doctors and pharmacists have a special duty of care and information about drugs that cause the disease.
The allergic variant requires quick action. This means administering risk-free antibiotics and stopping the causative agent. Older people and patients who take several risk substances are also at risk.
Further:
https://www.leukozyten-info.de/agranulozytose-verminderung-der-granulozyten.html
http://www.pharmazeutische-zeitung.de/index.php?id=29407
http://www.medical-tribune.de/medizin/fokus-medizin/artikeldetail/aufklaerung-ueber-agranulozytose-gefahr-verbessern.html
