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• W4387248237 abstract "SESSION TITLE: Diffuse Lung Disease Case Report Posters 12 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Lymphoplasmacytic lymphoma (LPL) is an uncommon B-cell lymphoproliferative neoplasm commonly involving the bone barrow, and sometimes lymph nodes and spleen. Pulmonary and mediastinal involvement in LPL has rarely been described. We present a patient with LPL diagnosed from mediastinal lymphadenopathy, interestingly associated with an interstitial lung disease (ILD). CASE PRESENTATION: A 66-year-old woman with history of MGUS underwent chest imaging after she suffered a manubrium sternum fracture following trivial trauma. She was incidentally found to have bilateral extensive patchy and confluent ground glass opacities with interlobular septal thickening and scattered air trapping. Bilateral hilar and mediastinal lymphadenopathy was also noted. Clinically, she was noted to be having worsening dyspnea on exertion over a period of months. PET scan only revealed diffuse low avidity heterogenous increase in metabolic activity at the areas of bilateral patchy ground glass opacities. Bronchoscopy with BAL, transbronchial biopsies, and EBUS guided transbronchial needle aspiration was performed. The initial results of biopsies and needle aspirates did not reveal evidence of malignancy, but flow cytometry subsequently revealed evidence of a B-cell lymphoproliferative disorder. This prompted a review of the original biopsy and aspirate samples, which revealed a MYD88 mutation, leading to diagnosis of lymphoplasmacytic lymphoma. She was referred to medical oncology, and is currently receiving multiple cycles of Rituximab and Bendamustine. An extensive rheumatological, immunological, and infectious investigation for the cause of her ILD was unrevealing. Open lung biopsy was offered but declined by the patient. Interestingly, repeat CT chest after two cycles chemotherapy showed significant improvement in the ground glass opacities, interstitial thickening, areas of air trapping, and the size of the lymphadenopathy. DISCUSSION: Lymphoproliferative disorders primarily affecting the lung are rarely observed. The spectrum of disorders include reactive pulmonary lymphoid diseases, Castleman disease, primary pulmonary lymphoma, and post-transplantation lymphoproliferative disorders. Lymphoplasmacytic lymphoma involving the lungs or mediastinal lymph nodes is even less common. Prominent interstitial lung disease has seldom been associated with pulmonary lymphoproliferative disorders. Our patient's primary imaging abnormality was confluent ground glass opacities with interlobular septal thickening. Initially, we thought that her ILD process was a separate entity, but no obvious cause for her ILD was found. Her medical, social, and environmental histories were unrevealing. We explored rheumatological (all serologies negative), immunological (hypersensitivity pneumonitis IgG panel, Southeastern regional allergen panel with IgE, Immunoglobulin levels, lymphocytic predominant BAL without eosinophilia), and infectious causes (negative cultures from BAL) for her ILD also. Her symptoms of shortness of breath improved once her chemotherapy regimen with Rituximab and Bendamustine. Surprisingly, her CT chest after two cycles of chemotherapy also revealed significant improvement in the ILD process. We believe this represents a rare presentation of interstitial lung disease, due to unusual pulmonary and mediastinal involvement from lymphoplasmacytic lymphoma. CONCLUSIONS: Keeping this patient's presentation in mind when faced with a supposed idiopathic interstitial pneumonia may open different avenues of investigation aimed at lymphoproliferative disorders as the underlying cause for interstitial lung disease. REFERENCE #1: Lymphoproliferative lung disorders: clinicopathological aspectsVenerino Poletti, Claudia Ravaglia, Sara Tomassetti, Carlo Gurioli, Gianluca Casoni, Silvia Asioli, Alessandra Dubini, Sara Piciucchi, Marco ChilosiEuropean Respiratory Review Dec 2013, 22 (130) 427-436; DOI: 10.1183/09059180.00004313 REFERENCE #2: Sanguedolce F, Zanelli M, Zizzo M, Bisagni A, Soriano A, Cocco G, Palicelli A, Santandrea G, Caprera C, Corsi M, Cerrone G, Sciaccotta R, Martino G, Ricci L, Sollitto F, Loizzi D, Ascani S. Primary Pulmonary B-Cell Lymphoma: A Review and Update. Cancers. 2021; 13(3):415. https://doi.org/10.3390/cancers13030415 REFERENCE #3: Kaseb H, Gonzalez-Mosquera LF, Parsi M, et al. Lymphoplasmacytic Lymphoma. [Updated 2022 May 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513356/ DISCLOSURES: No relevant relationships by Badri Giri No relevant relationships by David LeBel No relevant relationships by Aditya Sithamraju No relevant relationships by Elspeth Springsted" @default.
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• W4387248237 date "2023-10-01" @default.
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• W4387248237 title "UNIQUE PRESENTATION OF A DIFFUSE LUNG DISEASE IN A LYMPHOPROLIFERATIVE DISORDER" @default.
• W4387248237 doi "https://doi.org/10.1016/j.chest.2023.07.2082" @default.
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