Published online Jun 6, 2020. doi: 10.12998/wjcc.v8.i11.2305
Peer-review started: December 24, 2019
First decision: January 13, 2020
Revised: March 13, 2020
Accepted: May 13, 2020
Article in press: May 13, 2020
Published online: June 6, 2020
Processing time: 166 Days and 23.8 Hours
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%).
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.
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.
Core tip: Waldenström macroglobulinemia is a type of small lymphocytic lymphoma that mainly affects the bone marrow, spleen, and lymph nodes. Amyloidosis of the lower respiratory tract associated with Waldenström macroglobulinemia occurs very rarely. Here we report a patient who exhibited prominent pulmonary involvement. Chest computed tomography showed extensive multifocal and pleomorphic lesions in the patient's lungs. A computed tomography-guided percutaneous pulmonary biopsy confirmed the amyloid deposition.