Clinical analysis of eight patients with lung biopsy-proven pulmonary intravascular large B-cell lymphoma in a single center.

Objectives: The study aimed to describe the confirmed cases via lung biopsy to accurately reflect the characteristics and improve the prognosis of pulmonary IVLBCL patients.

Methods: We retrospectively reviewed medical records of patients with pathologically confirmed IVLBCL between July 2014 and December 2023. Patients diagnosed with pulmonary IVLBCL by lung biopsy were enrolled.

Results: Among 66 patients with IVLBCL, 8 patients (5 males and 3 females) were enrolled. The mean age was 54.6 ± 11.3 years old. Fever (87.5%), exertional dyspnea (75%), hypoxia (75%), and weight loss (>5 kg, 50%) were common clinical manifestations. Elevated lactate dehydrogenase (LDH, 100%), erythrocyte sedimentation rate (75%), C-reactive protein (75%), and hypoalbuminemia (75%) were common. Most patients showed diffuse ground-glass opacities (GGOs) with thickened interlobular septa on chest CT, highlighted by shadows on PET-CT. Bronchioalveolar lavage fluid analyses were usually normal; however, seven patients (87.5%) were identified as having IVLBCL via transbronchial lung biopsy. There were five patients using the regimen of R-CHOP or combined with Zanubrutinib, and one patient using Zanubrutinib-Rituximab. Finally, three patients died, four patients were in complete remission, and one was lost to follow-up. Among the three patients who died, one suffered from severe infection, and the other two suffered from lymphoma progression.

Conclusions: Patients with pulmonary IVLBCL mainly suffer from fever, weight loss, dyspnea, hypoxia, hyperinflammatory cytokine levels and elevated LDH levels. Diffuse GGOs and thickening interlobular septa with high uptake of ¹⁸F-FDG were common radiological features. Bronchoscopy might be an effective tool for the diagnosis of pulmonary IVLBCL.