Rafael Ríos-Tamayo, MD, PhD – Department of Hematology, University Hospital Virgen de las Nieves, Granada, Spain

Series: Recent Advances in Hematology Research; Rare Disorders Research Progress
BISAC: MED038000; MED014000; MED028000
DOI: https://doi.org/10.52305/FOJX1530

Systemic light chain (AL) amyloidosis is a complex, heterogeneous, multifaceted, rare, and challenging disease. Its origin is a bone marrow proliferation of clonal plasma cells, which secrete a toxic amyloidogenic immunoglobulin light chain. This monoclonal protein forms fibrils that finally deposit in key organs, like heart or kidney, causing organ failure. The book aims to offer a practical, critical, holistic and updated perspective of the disease, hopefully contributing to increase the awareness of this special entity. Every effort should be made to suspect the disease at early stages, avoiding diagnostic delay. Failure to achieve a timely diagnosis is associated with severe organ damage, which increases the risk of early death. The book is aimed at all audiences, including the entire range of medical specialties that are part of the multidisciplinary team that cares for these patients, health science students, researchers, pharmacists, patients, caregivers, and other. Five chapters cover the most relevant topics of general interest, underscoring as the knowledge about this entity has dramatically changed over the past decade. These areas are epidemiology, diagnosis, prognosis, treatment and the multidisciplinary approach in the setting of a well-developed amyloidosis unit. Epidemiological trends, technological diagnostic advances, new prognostic tools, emerging therapeutic approaches, the role of genomics and artificial intelligence, among other aspects, have been summarized, unveiling the underlying complexity of this disease. This book is the author’s own honest and humble tribute to patients with systemic AL amyloidosis, to give them a message of strength and optimism.