Primary pulmonary intravascular large B-cell lymphoma is a rare malignancy that can mimic interstitial lung disease on imaging, requiring high clinical suspicion and lung biopsy for accurate diagnosis.
Highlights fever and elevated LDH/CRP in rare pulmonary IVLBCL; leaves open prospective studies to refine diagnosis.
The present study aimed to investigate the clinicopathological features of primary intravascular large B-cell lymphoma (IVLBCL) of the lung. The clinical and histopathological data of three patients, and the literature was reviewed. The Ethics Committees of Drum Tower Hospital approved the current study based on the three cases. Fever and respiratory symptoms were the main presenting symptoms. Serum lactate dehydrogenase and C-reactive protein were significantly increased. Diffuse ground glass opacities or nodular consolidations were seen on high resolution computed tomography. Lung biopsy revealed lymphoma cells in the lumen of small blood vessels. Tumor cells expressed cluster of differentiation 20 and melanoma associated antigen (mutated) 1. Primary pulmonary IVLBCL is extremely rare and its prognosis is poor. Full recognition of its clinical character and improvement of the diagnostic awareness may help to reduce missed diagnosis, and facilitate appropriate treatment.
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Zhang et al. (2018) studied this question.
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