Diffuse Lung Disease |

Unusual Case of Cystic Lung Disease FREE TO VIEW

Salah Fares, MD; Daniel Dilling, MD
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Loyola University Medical Center, Maywood, IL

Chest. 2014;146(4_MeetingAbstracts):393A. doi:10.1378/chest.1990808
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SESSION TITLE: Interstitial Lung Disease Cases II

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PM

INTRODUCTION: The Differential diagnosis of cystic lung lesions is broad, it has been reported that cystic lung disease can a part of the of the spectrum of pulmonary lymphoid lesions, we are presenting a rare case of non-Hodgkin primary pulmonary lymphoma causing cystic lung lesions.

CASE PRESENTATION: A 64-year-old woman presented with shortness of breath. Chest CT revealed right lower lobe mass, bilateral pulmonary nodules, and multiple thin-walled cysts with lower lobe predominance. There was no adenopathy. She never smoked, did not have any significant occupational exposures, and had no unusual hobbies or pets. She had no personal history of lung disease or pneumothorax and no family history of tuberous sclerosis. Examination was unremarkable with normal oxygen saturation, and labs including CBC, ANA, anti-SSA , anti-SSB, VEGF-D were normal. she had bronchoscopy and subsequently a core-needle biopsy of the dominant mass. Histopathology demonstrated diffuse large B Cell Lymphoma representing 80% of the biopsy core, while 20% comprised low-grade B Cell Lymphoma with plasmacytic differentiation. Free kappa and lambda light chains were markedly elevated, and the cystic presentation with nodular deposits was determined to be a case of pulmonary light chain deposition disease (LCDD). PET scanning demonstrated Innumerable areas of increased metabolic activity with several subcutaneous soft tissue nodules throughout the body. She received six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone , which was tolerated well. Her shortness of breath improved, and follow-up PET/CT in 6 months showed resolution of the pulmonary hyper-metabolic nodules and stable cystic burden.

DISCUSSION: Primary pulmonary lymphoma is rare, however marginal-zone lymphomas compromise the majority cases.Our patient had multiple nodules and bilateral cysts, and we believe the cysts are a result of light chain deposition causing dilation of small airways. Others have suggested that amyloid deposition in the process of lymphocytic Infiltration can cause these cystic changes, although here there was no evidence for amyloid deposition.

CONCLUSIONS: the differential diagnosis of cystic lung disease includes is broad, light-chain deposition disease is a rare cause of cystic formation in the lung , and pulmonologists should have a keen awareness of all causes of cystic disease.

Reference #1: Parul Bhargava, MD,*w Jeanne M. Rushin, MD,z Eileen J. Rusnock, BS, and William D. Travis, MD, (Am J Surg Pathol 2007;31:267-276)

DISCLOSURE: The following authors have nothing to disclose: Salah Fares, Daniel Dilling

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