Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Plasma Cell Dyscrasias of the Lung: An Atypical Presentation of Non-IgM Lymphoplasmacytic Lymphoma FREE TO VIEW

Emilie Wang, MD; Angel Martin, MD; Nishan Tchekmedyian, MD; Gopi Prithviraj, MD
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H. Lee Moffitt Cancer Center and Research Institute/University of South Florida, Tampa, FL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1098A. doi:10.1016/j.chest.2016.08.1205
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Lymphoplasmacytic lymphoma (LPL) is an uncommon plasma cell dyscrasia. Non immunoglobulin M (IgM) LPL is seen in less than 5 percent of cases and rarely presents with pulmonary involvement. We describe a case of a 62 year-old man with non-IgM LPL that presented for symptoms consistent with hyperviscosity syndrome, malignant pleural effusion, and pneumothorax.

CASE PRESENTATION: A 62-year-old man with stage IV Non-IgM LPL presented to the oncology clinic for follow up. He endorsed dyspnea, headaches, visual disturbances, and ataxia. Due to concerns of hyperviscosity syndrome, he was admitted to the hospital for treatment. A computed tomography (CT) chest was performed which showed a large right pleural effusion with pleural thickening and small pneumothorax (Image 1). Cytology of the pleural fluid was consistent with LPL. These image findings were worse as compared to a staging positron emission tomography (PET)/CT scan three months prior which showed a small right pleural effusion with pleural thickening (Image 2). On admission, he was started on plasmapheresis with subsequent chest tube placement. A follow up chest X-ray revealed improvement of the right pleural effusion as well as the pneumothorax. Overall, his symptoms resolved and the chest tube was removed. He was discharged and is currently undergoing chemotherapy with rituximab, cyclophosphamide, and steroids.

DISCUSSION: The incidence of IgM LPL with pulmonary involvement has been reported as 3 to 5 percent (1). To our knowledge, this is one of the first reported cases of non-IgM LPL with pulmonary findings. Our patient developed recurrent pleural effusions and pneumothorax with persistent pleural thickening. Though pleural biopsies were not obtained, pleural effusion cytology was consistent with LPL, which may have predisposed him to these complications. Treatment of underlying disease with rituximab-based therapy has been the proposed intervention (2). Despite this, periodic thoracentesis, chest tube placements, and pleurodesis have also been utilized for these recurrent pulmonary manifestations due to poor pleural penetrance of chemotherapy (3).

CONCLUSIONS: A rare case of Non-IgM LPL with pulmonary involvement has been described. It remains uncertain if pulmonary involvement in these conditions affects overall disease prognosis. Though no definite guidelines regarding the best treatment approach has been reported, treating the underlying malignancy with chemotherapy still remains the standard of care.

Reference #1: Fadil, A. and D.E. Taylor, The lung and Waldenstrom's macroglobulinemia. South Med J, 1998. 91(7): p. 681-5.

Reference #2: Cao, X., et al., Clinicopathologic features and outcomes of lymphoplasmacytic lymphoma patients with monoclonal IgG or IgA paraprotein expression. Leuk Lymphoma, 2015: p. 1-10.

Reference #3: Amin, C.J. and I. Rabinowitz, An unusual reoccurrence of Waldenstrom's macroglobulinemia as pleural effusions that had a discordant response with treatment. Clin Lab Haematol, 2005. 27(3): p. 200-2.

DISCLOSURE: The following authors have nothing to disclose: Emilie Wang, Angel Martin, Nishan Tchekmedyian, Gopi Prithviraj

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