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Amyloidosis presenting as bilateral transudative pleural effusions with normal cardiac investigations: a case report

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      Abstract

      A 66-year-old man with a diagnosis of monoclonal gammopathy of unknown significance was referred for investigation of bilateral transudative pleural effusions by the cardiology team. Echocardiography, myocardial perfusion scanning and left heart catheterisation were all normal or non diagnostic. Given significant occupational asbestos exposure in his twenties he underwent thoracoscopic pleural biopsy. This showed fibrous inflammation only. He subsequently developed proteinuria and peripheral oedema. Reanalysis of the pleural biopsy specimen for amyloidosis was positive. Pleural disease is an uncommon presentation of systemic amyloidosis. The aetiology of the pleural effusions is unclear and is not simply a consequence of cardiac or renal impairment.

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      Most cited references 5

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      Amyloidosis and the respiratory tract.

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        Epidemiology and outcomes research for MGUS, myeloma and amyloidosis.

        The epidemiology of plasma cell dyscrasias clearly links to a complicated multi-factorial pathogenic pathway that at the individual patient level gives no clear indication of why the malignant process has occurred but factors in the environment and within the genome give clues and are discussed. MGUS is a pre-malignant disorder characterised by monoclonal plasma cell proliferation in the bone marrow and no end-organ damage; the patients are asymptomatic. Primary amyloidosis is a rare disorder that is characterised by deposition of amyloid fibrils composed of immunoglobulin light chain fragments; symptoms relate to the affected organ. Multiple myeloma is a malignant disease of plasma cells and with improvements in treatment, patients can now expect a doubling of median survival to 5 years, a 20% chance of surviving >10 years and a 50% chance of complete remission (CR), morphological and biochemical. The challenge is now to determine exactly what this means to the individual myeloma patient in terms of benefit, and to society as a whole and this is the basis of 'outcomes research' which is discussed in this review.
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          Persistent pleural effusions in primary systemic amyloidosis: etiology and prognosis.

          Restrictive cardiomyopathy frequently complicates primary systemic amyloidosis (AL), yet only a small number of these patients develop large pleural effusions refractory to diuretic therapy and thoracentesis. We hypothesized that disruption of pleural function by amyloid deposits underlies persistent pleural effusions (PPEs) in patients with AL disease. We performed a retrospective study of AL patients with and without PPEs who had been referred to Boston University between 1994 and 2001. The presence of PPEs was defined by a failure to resolve the condition with thoracentesis and aggressive diuresis. AL cardiomyopathy patients without pleural effusions constituted the control (cardiac) group. Indexes of plasma cell dyscrasia, nephrotic syndrome, thyroid function, and echocardiographic measures of left and right ventricle performance were compared between groups. When available, closed needle biopsies and autopsy specimens of parietal pleura were examined for amyloid deposits. Among 636 patients with AL, 35 PPE patients underwent a median of three thoracenteses each. No statistical differences were found between the PPE and cardiac groups in echocardiographic measures of septal thickness, left ventricular systolic function, or diastolic compliance. Right ventricular (RV) hypokinesis occurred more often in PPE patients; however, nearly half of this group had normal RV systolic function. Renal function, plasma protein levels, and thyroid function were the same between groups. Nephrotic range proteinuria (ie, > 3 g/d) was more prevalent in the cardiac group than in the PPE group (44% vs 26%, respectively; p = 0.057). All pleural biopsies in the PPE group (six biopsies) revealed amyloid deposits. Autopsy samples of parietal pleura were negative for disease in two cardiac patients. Eighteen patients had chest tubes placed, and 11 underwent pleurodesis. PPE signaled limited survival among patients who were ineligible for treatment. Untreated PPE patients lived a median 1.8 months vs 6 months for untreated cardiac patients (p = 0.031). Survival after intensive chemotherapy and autologous stem cell transplantation was comparable in the PPE and cardiac groups (21.8 vs 15.6 months, respectively; p = 0.405). In AL patients with cardiac amyloid, neither echocardiographic measures of ventricular function nor the degree of nephrosis distinguished those patients with PPEs. We conclude that pleural amyloid infiltration plays a central role in the creation and persistence of pleural effusions among patients with AL.
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            Author and article information

            Affiliations
            [1 ]simpleDepartment of Respiratory Medicine, Heatherwood and Wexham Park Hospitals NHS Foundation Trust, King Edward VII Hospital Windsor, Berkshire, SL4 3DPUK
            [2 ]simpleDepartment of Histopathology, Royal Brompton and Harefield NHS Trust Harefield, Middlesex, UB9 6JHUK
            Contributors
            Journal
            Cases J
            Cases Journal
            Cases Network Ltd
            1757-1626
            21 July 2009
            2009
            : 2
            2740277
            19829885
            10.4076/1757-1626-2-6963
            6963
            © 2009 Briggs et al.; licensee Cases Network Ltd.

            This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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            Medicine

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