Acquired Agranulocytosis - What Is It, Causes, and Treatment

Introduction

Agranulocytosis, often referred to as agranulosis or granulopenia, is an acute illness marked by a significant and potentially fatal decline in the number of white blood cells (leukopenia, most frequently of neutrophils), which results in neutropenia in the blood that is circulated. There is a substantial deficiency in one important class of white blood cells that fight infections. Because of their weakened immune systems, people with this illness have an extremely high risk of developing deadly infections.

What Is Acquired Agranulocytosis?

1. A substantial decrease in the quantity of white blood cells (granulocytes) in the bloodstream characterizes the uncommon drug-induced blood condition known as acquired agranulocytosis. The term "granulocyte" relates to the cell's grain-like structures. Basophils, eosinophils, and neutrophils are types of granulocytes.

2. Many medications have the potential to cause acquired agranulocytosis. Yet, certain pharmaceuticals used to treat cancer (cancer chemotherapeutic agents) and others used as antipsychotic medicines are among the medications to which a patient may be susceptible (for example, Clozapine). This disorder's symptoms are brought on by impairment in the bone marrow's ability to produce granulocytes.

3. Individuals who have developed agranulocytosis are vulnerable to a wide range of bacterial illnesses, which are often brought on by the body's normally benign bacteria. Very occasionally, the mucous membranes that border the mouth and/or gastrointestinal system can get painful ulcers.

What Are the Types and Causes of Agranulocytosis?

Two forms of agranulocytosis exist. Congenital means that one is born with the condition in the first category. The second kind is acquired. The patient with both types of agranulocytosis has a critically low neutrophil count. Adults with healthy levels typically have 1,500 to 8,000 neutrophils per microliter (mcL) of blood. Less than 500 cells per mcL are seen in agranulocytosis.

In acquired agranulocytosis, the bone marrow either fails to create neutrophils or produces neutrophils that do not develop into fully mature, functional cells. A factor that causes the neutrophils to deplete too fast is another possibility. Possible causes of acquired granulocytosis include:

1. Certain medicines.

2. Chemical exposure, such as that from the pesticide DDT.

3. Illnesses like cancer that impact the bone marrow.

4. Significant infections.

5. Radioactive contamination.

6. Systemic lupus erythematosus is one example of an autoimmune illness.

7. Nutritional inadequacies, such as low levels of folate and vitamin b-12.

8. Chemotherapy.

According to a 1996 study, drugs account for around 70 percent to 80 percent of the causes of acquired agranulocytosis. The following medicines can result in agranulocytosis:

1. Antithyroid Drugs like Methimazole and Carbimazole.

2. Nonsteroidal Anti-Inflammatory Medicines (NSAIDs), Sulfasalazine, and Metamizole are examples of anti-Inflammatory pharmaceuticals (NSAIDs).

3. Antipsychotic medications, such as Clozapine.

4. Quinine (an example of an antimalarial drug).

What Are the Symptoms of Acquired Agranulocytosis?

Depending on how severe the illness is, acquired agranulocytosis may present with a variety of non-specific symptoms. Typical signs include:

1. Fever.

2. Chills.

3. Unwell throat.

4. Oral sores.

5. Skin infections.

6. Breathing infections.

7. Digestive tract infections.

In addition to weariness and weakness, people with acquired agranulocytosis could also feel generally sick. It is crucial to get medical help right away if one has any of these signs since untreated agranulocytosis can cause life-threatening consequences.

How Is the Diagnosis of Acquired Agranulocytosis Done?

The conventional method for diagnosing acquired agranulocytosis is a mix of physical examination, blood testing, and medical history.

1. The first diagnostic test for agranulocytosis is a complete blood count (CBC) test, which counts the quantity of white blood cells, primarily neutrophils, in the blood. Agranulocytosis is characterized by a low white blood cell count, notably a low neutrophil count.

2. Other blood tests, such as blood culture, may be carried out to identify any bacterial, viral, or fungal infections that may be present in the body if the CBC test reveals a low white blood cell count. To confirm the diagnosis and obviate the presence of any other underlying disorders, a bone marrow biopsy may also be carried out.

3. The medical past has a significant role in determining the diagnosis of acquired agranulocytosis. Agranulocytosis can be brought on by specific drugs or chemicals; thus, the doctor may inquire about any recent exposure to these substances by the patient.

What Is the Long-Term Outlook for Agranulocytosis?

Since agranulocytosis renders the person sensitive to infection, it may be highly serious if it is left untreated. Sepsis is one of the side effects of agranulocytosis. It is an infection of the blood. Sepsis can lead to death if it is not treated. With prompt therapy, the outlook for agranulocytosis is improved. The condition can frequently be managed. Agranulocytosis caused by a viral infection may even go away on its own for some people.

What Is the Treatment of Acquired Agranulocytosis?

1. If at all feasible, the medicine or substance that is causing the acquired agranulocytosis must be stopped for therapy. This will shield the bone marrow from additional harm and enable it to heal and resume generating white blood cells.

2. If the patient develops a fever or other symptoms of illness, hospitalization may occasionally be necessary to treat and prevent infections. In order to prevent or treat infections, the patient may get intravenous antibiotics while in the hospital.

3. Moreover, drugs that promote the formation of white blood cells, such as granulocyte-colony stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor, may be recommended to patients with acquired agranulocytosis (GM-CSF). These medications help to increase the production of neutrophils in the bone marrow, which can help to reduce the risk of infection.

4. In severe cases of acquired agranulocytosis, blood transfusions may be necessary to replace the destroyed white blood cells with healthy ones.

How Is the Prognosis of Acquired Agranulocytosis?

The underlying cause and the severity of acquired agranulocytosis can both affect the prognosis. Many people recover in a few weeks or months with quick and effective therapy. But, in severe situations, problems such as sepsis and organ failure can ensue, which can be life-threatening.

How Is the Prevention of Acquired Agranulocytosis Done?

Although acquired agranulocytosis is an uncommon illness, it can be avoided by closely monitoring individuals using drugs that are known to cause it. To keep track of the white blood cell count and spot any irregularities early on, routine blood tests may also be carried out.

Conclusion

An uncommon medical disorder called acquired agranulocytosis can be brought on by exposure to specific drugs or substances. Low white blood cell counts, especially low neutrophil counts, which put people at risk for life-threatening infections, are the condition's hallmark. To avoid complications and enhance results, treatment must be administered promptly and appropriately. If a person encounters any signs of agranulocytosis, it is crucial to seek medical assistance immediately.