Lung Pathology: Student/Resident Case Report Poster - Lung Pathology I |

A Rare Case of Pulmonary Amyloidosis Secondary to Multiple Myeloma FREE TO VIEW

Umair Tariq, MD
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University of Miami Jackson Memorial Hospital, Miami, FL

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

Chest. 2016;150(4_S):803A. doi:10.1016/j.chest.2016.08.899
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SESSION TITLE: Student/Resident Case Report Poster - Lung Pathology I

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Amyloidosis(AM) is an extracellular tissue deposition of fibrils composed of variety of proteins and have been found to occur in roughly 10% of multiple myeloma (MM) patients. Pulmonary AM is a rare disease which is caused by extracellular deposition of fibrillary proteins. Pulmonary parenchyma is an uncommon site of extramedullary involvement in MM. Only isolated cases with histological data have been reported in the literature. In our case, the pulmonary AM was the initial presentation of MM.

CASE PRESENTATION: A 58 y/o male with no significant medical history presented with progressive dyspnea on exertion for about six months. Initial cardiac workup included an angiogram which showed non-obstructive coronary arteries with preserved left ventricular ejection fraction and elevated left ventricular diastolic pressures. Patient also had significant hypoxemia on admission, which prompted a contrast CT of chest negative for pulmonary embolism however showed significant bilateral pleural effusions. Initial etiology for his pleural effusions was attributed to his diastolic heart failure and he was started on diuretic therapy which decreased the pleural effusions tremendously. A few months later patient presented again to our institution with dyspnea due to recurrence of pleural effusions. His rheumotological and repeat cardiac work-up did not explain the reasoning for his recurrent pleural effusions. A thoracentesis was repeated three times to help with his dyspnea however he kept on reaccumulating the pleural fluid. Eventually the patient underwent a video-assisted thoracoscopy surgery for pleurodesis and a wedge lung biopsy. Pathology report was significant for pulmonary AM, which lead to an oncology referral for a bone marrow biopsy which confirmed a diagnosis of MM. Upon discharge from the hospital he was started on chemotherapy.

DISCUSSION: AM is a known rare complication associated with MM. The most commonly affected organs are the kidneys, heart, spleen, lymphnodes and the liver. Patients with MM have an increased risk of developing amyloidosis which increases mortality significantly compared to MM with the absence of AM. A Median survival for untreated patients is 13 months, and with the development of heart failure the survival duration decreases to less than 4 months.

CONCLUSIONS: Our case signifies a rare presentation of secondary pulmonary AM in a patient with MM. All efforts should be made by a clinician to obtain a tissue diagnosis in patients with parenchymal lung changes to establish this diagnosis. In our case the tissue pathology from lung biopsy lead to proper diagnostic work-up, and eventually appropriate diagnosis and therapy for the patient. To this date there are no described cases of primary pulmonary AM.

Reference #1: J.Berk, MD, A. Regan, MD and M Skinner MD: Pulmonary and tracheobronchial Amyloidosis

Reference #2: James P. Utz, MD; Stephen J. Swensen, MD; and Morie A. Gertz, MD : Pulmonary Amyloidosis. The Mayo Clinic Experience from 1980 to 1993

DISCLOSURE: The following authors have nothing to disclose: Umair Tariq

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