SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Light Chain Deposition Disease (LCDD) is a rare entity with variable clinical course. We present the case of LCDD in a patient with cryptogenic organizing pneumonia (COP).
CASE PRESENTATION: A 37-year-old female with biopsy proven COP presented with progressive dyspnea while on prednisone. Examination was suggestive of congestive heart failure. Chest x-ray showed cardiomegaly and pulmonary edema. BNP was 4097 pg/ml, BUN 105 mg/dl and creatinine 3.2 mg/dl. Transthoracic echocardiography showed diastolic dysfunction. Patient underwent right heart catheterization and despite diuresis there was insignificant change in hemodynamics (table). Suspecting restrictive cardiomyopathy an endomyocardial biopsy was performed that showed focal interstitial hyalinization negative for Congo Red stain. Bone marrow biopsy showed marked lymphoplasmacytosis and Κ: λ of 6: <1. Kidney biopsy with immunofluorescence showed diffuse linear basement membrane and granular mesangial staining for κ chain (Fig.1). A diagnosis of LCDD was established.
DISCUSSION: LCDD results from production of κ light chains by an abnormal clone of B-lymphocyte (1). Chains are granular and do not stain with Congo Red differentiating it from amyloidosis. Protein electrophoresis and biopsy with immunohistochemical staining for light chains confirms the diagnosis. Patients usually present in 5th or 6th decade. 50-60% of patients have an associated lymphoproliferative disorder, most commonly multiple myeloma. Treatment options include cytotoxic chemotherapy and autologous stem cell transplantation (1). Prognosis depends upon the number and extent of organ involvement, age, coexisting multiple myeloma and extra renal light chain deposition (2).
CONCLUSIONS: This case highlights the need to recognize restrictive cardiomyopathy that may be obscured by a previously diagnosed pulmonary condition. Our patient currently receives outpatient cytotoxic chemotherapy and has progressed to renal failure requiring hemodialysis.
Reference #1: Weichman K, Dember LM, Prokaeva T, et al. Clinical and molecular characteristics of patients with non-amyloid light chain deposition disorders, and outcome following treatment with high-dose melphalan and autologous stem cell transplantation. Bone Marrow Transplant. Sep 2006;38(5):339-43.
Reference #2: Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F Am J Kidney Dis. 2003;42(6):1154
DISCLOSURE: The following authors have nothing to disclose: Sidharth Jogani, Llewellyn Foulke, Marc Judson
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