Agranulocytosis

Synonyms in a broader sense

Granulocytopenia

definition

As Agranulocytosis is called one dramatic drop in the body's own immune cells, the Granulocytes, below 500 granulocytes per 1 microliter of blood.

The granulocytes are one Subgroup of white blood cells, of the Leukocytes.
The white blood cells are that Carrier of our immune system, so the body's own defenses.
In addition to the granulocytes, there are also those Lymphocytes as a subgroup of white blood cells, however, they react more slowly to threats than granulocytes, which is why granulocytes are more important in the Defense against bacteria or at sudden inflammatory reactions.
A less severe form of granulocyte reduction is called Granulocytopenia.
The reason for agranulocytosis is often a drug cause.

The most important Medicationthat can cause agranulocytosis as a side effect are, for example, some pain relievers such as Metamizole®, nonsteroidal anti-inflammatory drugs such as. ASS (aspirin), the anticoagulant Ticlopidine or Antibiotics from the group of the sulfonamides such as e.g. Sulfamethoxazole. Neuroleptics or Anti-thyroid drugs are also considered possible triggers.
These drugs can interact with naturally occurring proteins in the blood (Plasma proteins) connect. The body mistakenly recognizes these compounds (complexes) made up of the drug substance and plasma protein as a threat and begins what is known as antibody to produce to mark the supposed enemy in the body for degradation.

These antibodies should ideally only adhere to the surface of the complex perceived as a threat. Unfortunately, the interface is similar to the the body's own innate defense cells, the granulocytes, the surface of the Drug-plasma protein complexes, which is why the antibodies that are formed mistakenly attach themselves to the surface of the granulocytes.
As soon as a Cell marked with antibodies is, the body begins with everyone Defense reaction, Phagocytes send out to combat them and also completely dismantle them. Because of a misinterpretation of the threat, the body begins to fight itself by creating its own, innate immune system attacks.

This form of agranulocytosis is called Type 1 agranulocytosis and often occurs as a sudden (acute) clinical picture.

Another, less acute cause of agranulocytosis can be Damage to the bone marrow be what the real The formation of granulocytes is impeded becomes.

As examples are here Bone marrow tumors to call or toxic damage of the bone marrow after a chemotherapy or various drugs.

In these forms, in addition to agranulocytosis, there is also one Pancytopenia before, i.e. a general reduction in the important cells that are formed in the bone marrow, including the red blood cells (Erythrocytes) and platelets (Platelets).

If pancytopenia is present, the cause must be investigated more closely by e.g. a Bone marrow sampling.

diagnosis

A Agranulocytosis is through a Analysis of the composition of the blood noted in the so-called Differential blood count. To acquire the material for this is only one simple blood collection necessary.

The number of granulocytes is noticeable below 500 cells per microliter of blood, one speaks of agranulocytosis.

Furthermore, it must be asked and clarified which medication the patient is taking or has taken. In addition, samples from the bone marrow can be useful for diagnostics in order to find out what is the cause of agranulocytosis.

Symptoms

Since granulocytes are part of the Immune system the symptoms are similar to the symptoms of a strong immunocompromised patients, for example patients with AIDS, Bone marrow tumor patients, Leukemia patients etc.

Immunocompromised patients are more prone to bacterial and viral infections, as well as to Fungal diseases (Mycoses). Not only do they get it easier, but they are also less able to fight the disease that has broken out.
You start to feel uncomfortable and develop flu-like symptoms how a headache, Joint pain, Loss of appetite, fever and chills. Since the mucous membranes around body entry ports, i.e. various body openings, are particularly susceptible to infections, these are also the first to become infected as soon as the immune system can no longer adequately protect them.

As typical Triad the disease are so fever, Tonsillitis (Angina tonsillaris) and Inflammation of the mouth with whitish deposits (Aphthous stomatitis) listed.

The body is usually able to contain inflammation and prevent it from spreading. If he fails to do this because his immune system is weakened, it is possible that bacteria or fungi will find their way into the bloodstream and become infected Inflammation spreads throughout the body, the patient becomes septic. A sepsis can be left untreated fatal end, especially in patients who have been weakened before.

therapy

Is the Agranulocytosis by Medication triggered, becomes the causative Drug discontinued and replaced by an alternative drug that does not have agranulocytosis as a side effect. The body should then be able to regain itself regenerate.

If a patient is already sick, it has to External immune system support get to fight the disease. It will Antibiotics and Antimyotics (Antifungal agents) used to help the weakened immune system fight the disease.
Affected can by increased hygiene measures and active avoidance of sources of infection take care to prevent infections.