Abstract

An 88-year-old woman with no significant medical history was admitted to the emergency room with persistent dyspnea and cough. Chest computed tomography (CT) showed diffuse ground-glass opacities in both the lungs (Fig. 1A). Collagen disease and infection were ruled out, and lymphoproliferative disease was suspected based on high level of WBC 15 200/μL (lymphocyte 63.7%), soluble interleukin 2 receptor 3070 (151–474) IU/mL and LDH 421 (115–245) U/L. Fluorodeoxyglucose (FDG) did not accumulate in the lymph nodes throughout the body, and its accumulation as ground-glass shadows (Fig. 1B) led us to suspect a lung lesion; therefore, we performed a bronchoscopy. The patient experienced severe hypoxemia during bronchoalveolar lavage, and the biopsy was cancelled. Cell block of bronchoalveolar lavage fluid showed CD20-positive atypical lymphocytes and plasma cells, which were κ chain-positive, IgM-positive, IgG-, IgA- and λ chain-negative (Fig. 2). Blood protein fractionation showed M-protein, with increased IgM at 679 (46–260) mg/dL, and decreased IgG at 425 (870–1700) mg/dL and IgA at 8 (110–410) mg/dL. Based on these findings, the patient was diagnosed with Waldenström macroglobulinemia (WM). The patient was treated with steroids, and fever and dyspnea symptoms were relieved. The haematologist performed a bone marrow biopsy (Fig. 3), and confirmed the diagnosis of WM based on the findings of the bone marrow biopsy and flow cytometry of peripheral blood, which showed small B cells with a κ > > λ bias, negative CD5 and CD23, and IgM-κ and Bence Jones protein-κ detected by immunofixation, after which the patient received rituximab. On chest CT 120 days after starting rituximab, the ground-glass opacities in the both lungs disappeared (Fig. 4), and there has been no relapse for more than a year to date.

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