Primary (AL) Amyloidosis in Plasma Cell Disorders

Abstract

Abstract Learning Objectives After completing this course, the reader will be able to: Describe the pathogenesis of AL amyloidosis.Identify other disorders often associated with AL amyloidosis.Discuss the importance of the prompt diagnosis and treatment of AL amyloidosis and any underlying plasma cell disorder.Identify presenting signs and symptoms that should lead to the suspicion of AL amyloidosis. Access and take the CME test online and receive 1 AMA PRA Category 1 Credit™ at CME.TheOncologist.com Primary (AL) amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain (LC) fibrils in major organs, such as the kidneys, heart, and bowel. Organ dysfunction contributes to a high mortality and poor prognosis, with a median survival time of 1–2 years from diagnosis. Here, we present a 46-year-old man with an exceptional clinical course of an LC multiple myeloma with generalized amyloidosis, causing renal insufficiency, congestive heart failure, and complete intestinal necrosis. We have summarized recent knowledge on AL amyloidosis, its association with monoclonal gammopathies, clinical presentations, diagnostic tools, and treatment strategies. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL amyloidosis. This may facilitate earlier diagnosis, and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.