Chest
Clinical InvestigationsPLEURAPersistent Pleural Effusions in Primary Systemic Amyloidosis: Etiology and Prognosis
Section snippets
Patients
We reviewed the records of patients referred to the Amyloid Research and Treatment Program at Boston University Medical Center between 1994 and 2001 for the treatment of AL. A diagnosis of AL required demonstration of amyloid deposits by Congo red staining and detection of monoclonal Ig protein in serum, urine, bone marrow, or tissue biopsy specimen. PPEs were defined as large pleural effusions seen on a chest radiograph (occupying one third or more of the hemithorax) that persisted despite
Results
Between 1994 and 2001, 636 patients with AL were referred to the Amyloid Treatment and Research Program at Boston University Medical Center for evaluation. Thirty-five patients (6%) had large pleural effusions on screening chest radiographs that failed to resolve with direct drainage and aggressive diuresis. Five patients with PPEs had multiple myeloma, and one patient had Waldenstro¨m macroglobulinemia. One hundred twenty patients with cardiac amyloid infiltration in the absence of pleural
Discussion
Our retrospective analysis identified two statistically significant differences between AL patients with PPE and those with AL cardiac disease and no pleural effusions (ie, the cardiac group), as follows: (1) more frequent RV hypokinesis in the PPE group; and (2) greater proteinuria in patients without effusions. Multiple echocardiographic measures of LV function failed to distinguish PPE patients from those with AL cardiomyopathy alone. Taken together, the frequency of exudative pleural fluid
ACKNOWLEDGMENT
We thank Arquimedes J. Areche for expert database management, Sandra A. Cerda, MD, and Niall Swan, MD, for the histologic preparation of pleural specimens.
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Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail:[email protected]).
This research was supported by grants from the National Institutes of Health (HL 68705), the US Food and Drug Administration (FD-R-001346), the Gerry Foundation, the Young Family Amyloid Research Fund, the Sue Sellors Finley Cardiac Amyloid Research Fund, the Amyloid Research Fund at Boston University, and the McCaleb Foundation. Dr. Seldin is a scholar of the Leukemia and Lymphoma Society of America.