Abstract 14545: Extramedullary Plasmacytoma of Tonsil Presenting as Cardiac Amyloidosis

Abstract

Introduction: AL amyloidosis is a systemic disease characterized by the deposition of misfolded immunoglobulin light chains produced from clonal plasma cells in multiple organs of the body causing its dysfunction. We present you an unusual case of AL cardiac amyloidosis in a patient with extramedullary plasmacytoma of the tonsils. Case Presentation 61-year-old female presented with dyspnea on exertion with NT-proBNP elevated to 1439 pg/ml. CXR showed increased pulmonary vascular congestion. The patient was started on IV furosemide 40 mg BID with good urine output. Transthoracic echocardiogram revealed left ventricular hypertrophy with an ejection fraction of 60-65% with a global longitudinal strain of -9.0% and regional longitudinal strain reduced in the basal and mid-left ventricular segment but preserved in apical segment consistent with infiltrative cardiomyopathy. The patient underwent a nuclear (nuclear technetium pyrophosphate) scan of the heart consistent with cardiac amyloid deposition. She also was noted to have increased tonsillar mass which was biopsied. In the hospital, the patient had a cardiac arrest with the initial rhythm being ventricular fibrillation. The patient received 6 rounds of shock, and multiple injections of epinephrine, amiodarone, and bicarbonate push. The patient died despite all efforts of resuscitation. Histopathology results of tonsillar mass were consistent with plasmacytoma with amyloid deposition on Congo-red stain. Lambda: Kappa ratio was 70:1. Discussion: In cardiac amyloidosis, light chain deposits between cardiac myocytes disrupt cell-cell interaction. It can present as restrictive cardiomyopathy, conduction system abnormalities, or sudden cardiac death. It is essential to diagnose cardiac amyloidosis earlier. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Conclusion: Our case was unusual in which a rare tumor of tonsils had plasma cell tumor which led to cardiac amyloidosis