Abstract
BACKGROUND: Waldenström macroglobulinemia (WM) is a type of small lymphocytic lymphoma that mainly affects the bone marrow, spleen, and lymph nodes. A subset of patients with WM demonstrates extramedullary involvement (4.4%), and the most frequent extramedullary disease site involved is the lungs (30%). CASE SUMMARY: A 60-year-old male patient who experienced intermittent breath-holding for 6 mo was admitted on August 14, 2017. Chest computed tomography indicated multiple pulmonary cavities in the upper lobes of both lungs, with pulmonary consolidation, ground-glass opacities, patchy infiltrates, fibrous bands, large bullae, and enlarged lymph nodes in the mediastinum. The patient was a heavy smoker (20 cigarettes/d for 40 years). Diagnostic fiberoptic bronchoscopy revealed normal findings. Serological examination revealed a remarkable increase in serum immunoglobulin M levels (30.24 g/L; normal: 0.4-2.30 g/L). A computed tomography-guided percutaneous pulmonary biopsy was performed in the left lower lobe of the lung with pulmonary consolidation and indicated that the alveolar structure disappeared and that a large amount of amyloid-like deposition was present along with the infiltration of very few lymphocytes and plasma cells. The patient was treated with the combined treatment of dexamethasone + rituximab + lenalidomide over four courses. Serum immunoglobulin M did not normalize, and he received ibrutinib + dexamethasone. CONCLUSION: This patient with WM and lung amyloidosis had a wide range of pulmonary lesions and a variety of morphological features, which was a rare case. Yet, some changes might be ascribed to heavy smoking.