Clinico-Pathological Conference

Abstract

A 53-year-old man was admitted to Keio University Hospital because of serious dyspnea and edema of the lower extremities. Eighteen months previously, the patient had complained of chest discomfort, and was then admitted for the first time to our hospital for evaluation of chest pain. Electrocardiography showed poor R wave progression in leads Vl through V4, and diffuse nonspecific STsegment and T wave abnormalities with low voltage. However, no definitive diagnosis could be made at this initial admission and a calcium-channel blocker was prescribed. Despite this treatment, the patient was readmitted with worsening dyspnea and lower extremity edema. The diagnosis of heart failure and nephritic syndrome was made at the second admission. In addition, immunoelectrophoresis showed a monoclonal IgD (l) M protein and increased plasma cells in the bone marrow, suggesting a diagnosis of multiple myeloma. The patient was thus given dexamethasone (20 mg per day for 4 days) intravenously, but his symptoms did not improve. Two weeks later, the patient deteriorated further with congestive heart failure and renal failure, and subsequently died of cardiac arrest with ventricular fibrillation. On autopsy, IgD (l)-positive plasma cell proliferation was found in the bone marrow, confirming the diagnosis of multiple myeloma. In addition, amyloid deposition was detected in various organs including the heart, kidneys, esophagus, duodenum, ileum, colon, tongue, and lungs. In particular, the weight of the heart was 650 g demonstrating a hypertrophic septum and amyloid deposition in the myocardium and even coronary arteries. In summary, the final diagnosis was IgD (l) multiple myeloma associated with systemic amyloidosis. (Keio J Med 53 (3): 178–191, September 2004)