From the Guidelines
Agranulocytosis is a medical emergency that requires immediate discontinuation of the causative agent, typically a medication, and prompt treatment with broad-spectrum antibiotics and granulocyte colony-stimulating factors to prevent life-threatening infections and promote neutrophil recovery. The condition is characterized by a severe reduction in neutrophils, a type of white blood cell essential for fighting infections, and can be caused by various factors including medication reactions, chemotherapy, radiation therapy, autoimmune disorders, and certain infections 1. Patients with agranulocytosis often present with fever, sore throat, mouth ulcers, and recurrent infections, and require careful monitoring of blood counts and supportive care to prevent complications.
Diagnosis and Treatment
The diagnosis of agranulocytosis is based on a complete blood count (CBC) showing a neutrophil count below 500 cells/mm³. Treatment involves:
- Immediately stopping the suspected causative agent
- Providing broad-spectrum antibiotics for infections, such as piperacillin-tazobactam or meropenem plus vancomycin for febrile neutropenia
- Administering granulocyte colony-stimulating factors like filgrastim (5-10 μg/kg/day subcutaneously) to stimulate neutrophil production
- Implementing supportive care, including isolation precautions to prevent infection, nutritional support, and careful monitoring of blood counts
Medication-Induced Agranulocytosis
Certain medications, such as clozapine, deferiprone, and antithyroid drugs, can increase the risk of agranulocytosis. The risk of agranulocytosis with deferiprone is approximately 1% of treated patients, and regular monitoring of neutrophil counts is essential to detect early signs of the condition 1. If agranulocytosis or neutropenia occurs, the medication should be stopped immediately, and patients should contact their physician.
Outcome and Prognosis
Prompt recognition and treatment of agranulocytosis are crucial to prevent life-threatening infections and promote neutrophil recovery. Recovery typically occurs within 1-3 weeks after removing the causative agent and implementing appropriate treatment. However, agranulocytosis carries a mortality rate of 5-10% if not addressed quickly, highlighting the importance of early diagnosis and treatment 1.
From the Research
Diagnosis of Agranulocytosis
- Agranulocytosis is characterized by a peripheral neutrophil count < 0.5 x 10(9)/l 2
- It often manifests with a severe sore throat, but isolated fever, pneumonia or septicaemia are not uncommon 2
- Frequent cell count monitoring is an essential tool to make a timely diagnosis 3
Treatment of Agranulocytosis
- Suspect drugs should be stopped immediately 2
- In febrile patients, blood cultures and, where indicated, site-specific cultures should be obtained and treatment with empirical broad spectrum antibiotics started 2
- Haematopoietic growth factors, such as granulocyte colony-stimulating factor (G-CSF), should be considered in patients with poor prognostic factors (e.g., a neutrophil count < 0.1 x 10(9)/l), severe clinical infection or severe underlying disease or comorbidity 2, 4, 5, 6
- G-CSF has been shown to be effective in reducing the duration of neutropenia and improving patient outcomes 4, 5, 6