Lung Pathology: Fellow Case Report Poster - Lung Pathology |

Not Your Average Neoplasm: Pulmonary Lymphomatoid Granulomatosis FREE TO VIEW

Pujan Patel, MD; Abhishek Krishna, MD; David Stoeckel, MD
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Saint Louis University Hospital, Saint Louis, MO

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

Chest. 2016;150(4_S):778A. doi:10.1016/j.chest.2016.08.874
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SESSION TITLE: Fellow Case Report Poster - Lung Pathology

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: Recognized in 1972, lymphomatoid granulomatosis is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder (LPD) with less than 600 cases reported in the literature. As a consequence, its diagnosis and optimal management remain a challenge despite advances in our understanding of the disorder.

CASE PRESENTATION: A 45 year old caucasian male mechanic with a 24 pack year history of prior smoking initially presented to an external facility with complaints of a fevers, night sweats, unintentional weight loss and a generalized macular rash with diffuse joint stiffness. Biopsy of the rash revealed lymphohistiocytic vasculitis and steroid treatment was initiated. His symptoms temporarily abated but a month later he represented for exertional dyspnea, mild hypoxia and an occasionally productive cough. Chest computed tomography revealed diffuse bilateral nodular opacities with a basilar predominance. Bronchoscopic evaluation was negative for infection and transbronchial biopsies were negative for malignancy. An extensive autoimmune and infective serologic work up was unrevealing. Persistence of symptoms prompted an open lung biopsy (image 2).

DISCUSSION: Typically seen in males between the ages of 30 and 50, lymphomatoid granulomatosis predominantly involves the lungs, skin, CNS, liver and kidneys. There is a known predilection towards immunodeficient patients given the impaired ability to mount an adequate T-cell mediated response to the EBV infection that it is known to be assciated with. The spleen, lymph nodes and bone marrow are usually spared. Hallmark features consist of atypical large EBV-positive B-cells with angiocentric and angiodestructive lymphocytic infiltrate.

CONCLUSIONS: Despite being aware of this entity and its clinical manifestations, histopathology remains central to diagnosis here. It is histopathology that will establish grade and hence guide management. Although there is no definitive consensus on management for each grade, responses have been seen with corticosteroids, interferon-alfa-2b, immunochemotherapy and stem cell transplantation in some. Five year mortality remains high at 53% -64% and so there is clearly room for advancement of its management.

Reference #1: Song JY, Pittaluga S, Dunleavy K, et al. Lymphomatoid granulomatosis—a single institute experience: pathologic findings and clinical correlations. Am J Surg Pathol. 2015;39:141-156.

Reference #2: Katzenstein AL, Doxtader E, Narendra S. Lymphomatoid granulomatosis: insights gained over 4 decades. Am J Surg Pathol 34:e35-e48, 2010.

DISCLOSURE: The following authors have nothing to disclose: Pujan Patel, Abhishek Krishna, David Stoeckel

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