Agranulocytosis (Neutropenia)

Agranulocytosis (Neutropenia) – Treatment Options

Introduction
Agranulocytosis is a severe and dangerous form of neutropenia, defined as an absolute neutrophil count (ANC) < 500 cells/µL, leading to a markedly increased risk of infection. It may arise from drug-induced bone marrow suppression (e.g., clozapine, carbimazole, sulfonamides, chemotherapy), autoimmune processes, bone marrow failure syndromes, or severe infections. Patients often present with fever, sore throat, mouth ulcers, pneumonia, or septicemia. Rapid recognition and intervention are essential to prevent fatal complications.

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1. Immediate Supportive Management

• Hospitalization and isolation: Protective/reverse isolation to reduce infection risk.

• Empiric broad-spectrum antibiotics: Initiated promptly at the onset of fever or suspected infection.

  • Choices include cefepime, piperacillin–tazobactam, or meropenem.

  • Add vancomycin if catheter-related or resistant Gram-positive infection is suspected.

  • Antifungal therapy (voriconazole, amphotericin B, or echinocandin) if fever persists >4–7 days despite antibiotics.

• IV fluids and hemodynamic support in septic patients.

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2. Hematopoietic Growth Factors

• Granulocyte colony-stimulating factor (G-CSF; filgrastim, lenograstim):

  • Accelerates neutrophil recovery.

  • Reduces infection-related complications, especially in chemotherapy- or drug-induced agranulocytosis.

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3. Removal of the Underlying Cause

• Drug-induced agranulocytosis:

  • Immediate discontinuation of the offending agent (e.g., clozapine, carbimazole, dapsone).

  • Re-exposure must be permanently avoided.

• Autoimmune agranulocytosis:

  • May require immunosuppressive therapy (e.g., corticosteroids, IV immunoglobulin).

• Bone marrow disorders (e.g., aplastic anemia, leukemia):

  • Managed with disease-specific therapies (immunosuppression, chemotherapy, or stem cell transplant).

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4. Advanced and Curative Options

• Hematopoietic stem cell transplantation (HSCT):

  • Considered in congenital severe neutropenia or refractory aplastic anemia.

• Granulocyte transfusions:

  • Rare, used as a rescue measure in life-threatening infections not responding to antibiotics and G-CSF.

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5. Supportive and Preventive Measures

• Oral care: Antiseptic rinses to reduce risk of mucosal infections.

• Antimicrobial prophylaxis: Fluoroquinolones, antifungals, or antivirals in high-risk patients with prolonged neutropenia.

• Vaccinations: Influenza and pneumococcal vaccines to prevent secondary infections.

• Nutritional support and management of comorbid conditions to improve immune resilience.

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6. Long-Term Monitoring

• Regular complete blood counts (CBCs) until recovery.

• Documentation of causative drugs to prevent future exposure.

• Monitoring for complications such as pneumonia, septicemia, or invasive fungal disease.