Diffuse Lung Disease |

Primary Pulmonary Lambda Light Chain (AL) Amyloidosis With Giant Cell Reaction Misdiagnosed as Sarcoidosis FREE TO VIEW

Jilalu Kelbe, MD; Linus Santo Tomas, MD; Nagarjun Rao, MD
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Medical College of Wisconsin, Wisconsin, WI

Chest. 2015;148(4_MeetingAbstracts):379A. doi:10.1378/chest.2242881
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SESSION TITLE: Diffuse Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary pulmonary AL amyloidosis is a rare entity caused by deposition of amyloid light chains in the lungs. Sarcoidosis is a systemic disease of unknown etiology which mainly affects the lungs and is pathologically characterized by non caseating granuloma. There are a few reports of pulmonary amyloidosis due to chronic inflammation from sarcoidosis. We report a case of primary pulmonary AL amyloidosis that was misdiagnosed as sarcoidosis with secondary amyloidosis.

CASE PRESENTATION: A 44-yr-old nonsmoker female patient presented with a few years of progressive dyspnea without cough, fever or other systemic symptoms. On examination, she had bilateral basal crackles. Her oxygen saturation was 93% on room air. Computerized tomography (CT) scan of the chest showed bilateral ground glass opacities and mosaic attenuation with a nodule at the left upper lobe. Complete blood count and basic chemistry tests were unremarkable. Pulmonary function test (PFT) revealed moderately severe obstructive defect with moderate diffusion block. Video assited thoracoscopic (VATS) lung biopsy was performed which showed a granulomatous inflammation with deposition of amyloid. A presumptive diagnosis of sarcoidosis was made and she was treated with prednisone. It was noted that she had subjective improvement at higher dose of prednisone but symptoms worsened when she was weaned off. Subsequent review of the slides confirmed that she had AL amyloid (lambda) deposition with foreign body type granulomatous reaction associated extranodal marginal cell lymphoma. Evaluation for systemic amyloidosis including abdominal fat aspirate and bone marrow examination were negative. Patient was started on rituximab.

DISCUSSION: Localized pulmonary AL amyloidosis is rare but can occur in the setting of pulmonary lymphoid malignancies with plasma cell differentiation. A giant cell type granulomatous reaction towards the amyloid fibrils may be seen and this may be mistaken for sarcoidosis which may also cause secondary amyloidosis.

CONCLUSIONS: Proper characterization of the amyloid fibrils is essential for the correct diagnosis of the underlying disease when features of both amyloidosis and granulomatous inflammation coexist in patients with primary pulmonary AL amyloidosis.

Reference #1: Ben Abdelghani K et al AA amyloidosis complicating sarcoidosis: 2 cases & literature review. Rev Med Interne; 2010 May;31(5):369-71

Reference #2: Xu, L et al Isolated pulmonary amyloidomas: report of 3 cases with histologic & imaging findings. Pathol Res Pract 2013 Jan 15;209(1):62-6

DISCLOSURE: The following authors have nothing to disclose: Jilalu Kelbe, Linus Santo Tomas, Nagarjun Rao

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