Diagnosis and management of systemic light chain AL amyloidosis

Abstract

AL amyloidosis is a plasma cell disorder leading to the production and extracellular deposition of abnormal immunoglobulin light chains called amyloid. The pathogenesis of the disorder is driven by an abnormal plasma cell clone producing excessive monoclonal immunoglobulin light chains that undergo deposition in various organs of the body such as the heart, kidney, and gastrointestinal tract. The outcome of the disease remains poor with significant morbidity and mortality associated with organ dysfunction. In this review, we describe the current standard diagnostic features, prognosis, and current treatment paradigm of the disease.

Keywords: AL amyloidosis; Diagnosis; Management; Staging.