[Pulmonary infection in neutropenia]

Area of Science:

• Hematology
• Mycology
• Oncology

Background:

• Hairy cell leukemia (HCL) is a rare B-cell malignancy.
• Treatment-refractory HCL poses significant management challenges.
• Patients with prolonged neutropenia are susceptible to opportunistic infections.

Observation:

• A 42-year-old patient with relapsed HCL developed shortness of breath and chest pain.
• Imaging revealed bilateral infiltrates and pleural effusions, suspicious for fungal infection.
• The patient experienced severe cytopenias, respiratory failure, and died despite aggressive treatment.

Findings:

• Diagnosis confirmed relapsed HCL with BRAF mutation and >80% bone marrow infiltration.
• Autopsy showed disseminated fungal hyphae in lungs and spleen, with hepato-splenomegaly and pancytopenia.
• Aspergillus antigen was detected and cleared with antifungal therapy, but septic complications led to death.

Implications:

• Refractory HCL with neutropenia necessitates early consideration of prophylactic antifungal therapy.
• Vemurafenib represents a potential therapeutic option for treatment-refractory HCL.
• This case highlights the critical risk of systemic fungal infections and the challenges in managing HCL complications.