Mysterious loss of white blood cells: How does agranulocytosis occur?

Agranulocytosis, also known as agranulocytosis or granulocytopenia, is an acute condition involving a marked decrease in the white blood cell count (too few leukocytes, most commonly neutrophils). This condition results in insufficient numbers of neutrophils circulating in the blood, putting patients at high risk for serious infections.

In agranulocytosis, the granulocyte concentration is less than 200 cells per mm3 of blood.

Symptoms and manifestations

Agranulocytosis is sometimes asymptomatic, but in other cases it manifests suddenly with symptoms such as high fever, chills, and sore throat. Infections in any organ can get worse quickly, such as pneumonia or a urinary tract infection. The most serious condition is sepsis, which can also progress very quickly.

Causes

Agranulocytosis can occur for many reasons, including some medications. Antiepileptic drugs (such as carbamazepine and valproic acid), antithyroid drugs, certain antibiotics (such as penicillin and chloramphenicol), H2 receptor antagonists, ACE inhibitors, and some antidepressants and antipsychotics may cause this situation.

In particular, the use of the atypical antipsychotic drug clozapine is strictly restricted, with mandatory monitoring of its users' blood counts.

In addition, the Centers for Disease Control and Prevention tracked an outbreak of agranulocytosis in Coca-Cola users in the United States and Canada in 2008-2009, primarily due to the presence of rivanorphine in cocaine drugs.

Diagnostic Process

Diagnosis of agranulocytosis usually requires a complete blood count. In this test, the absolute neutrophil count will be less than 500 and may even reach 0. To formally diagnose agranulocytosis, other conditions with similar presentations, such as aplastic anemia and leukemia, need to be ruled out, which usually requires a bone marrow examination.

Examination of the bone marrow showed normal numbers and types of cells, but hypoplastic progranulocytes were found.

Classification of Agranulocytosis

The term agranulocytosis comes from the Greek word meaning "without granule cells." However, a complete absence of these cells is not required for diagnosis. Agranulocytosis is more significant than granulocytopenia, which refers to an insufficient number of granulocytes, while agranulocytosis is more severe and is usually defined as a neutrophil count of less than 100 per microliter.

The pathological evolution of agranulocytosis can be divided into two main categories: inappropriate or ineffective granulocyte formation or accelerated neutrophil destruction.

Treatment options

If the patient has no symptoms of infection, treatment strategies focus on close monitoring with regular blood counts, discontinuation of the offending medication, and counseling about the significance of the high fever. Although granulocyte transfusion has been proposed as a solution, its effect is very short-lived because granulocytes survive in the circulation for only about 10 hours.

Such a treatment regimen still presents a risk of agranulocytosis. Even with the improvements, how to effectively prevent and manage this condition remains a major challenge for the medical community. What kind of progress will future research bring to solve this mysterious lack of white blood cells?

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