Understanding Amyloidosis: Unraveling the Complexities and Therapeutic Approaches for Oncology Nurses.

BACKGROUND:  Primary systemic light-chain (AL) amyloidosis is a rare clonal plasma cell disorder characterized by the production of abnormal immunoglobulin fragments, which form insoluble fibrils that aggregate as amyloid deposits in organs and tissues, leading to organ dysfunction and death. OBJECTIVES:  The aim of this literature review is to increase awareness of AL amyloidosis and educate nurses on the care of this patient population. METHODS:  This overview is based on a literature search of AL amyloidosis, including its pathogenesis, prognosis, and presentation. Guidance for nursing assessment, intervention, and patient education throughout the disease trajectory is presented. FINDINGS:  AL amyloidosis is a rare disease resulting in organ impairment and death if untreated. Nursing management includes knowledge of key assessment, monitoring, intervention, and education strategies with goals to preserve organ function and improve survival and quality of life in patients with AL amyloidosis.

Immunoglobulin Light-Chain Amyloidosis: Clinical Presentations and Diagnostic Approach.

Systemic immunoglobulin light-chain (AL) amyloidosis is a rare disorder arising from a plasma cell clone that produces misfolded immunoglobulin light chains, which are deposited in various tissues and organs as amyloid fibrils. Signs and symptoms are typically vague and overlap with those arising from other common diseases; consequently, diagnosis of AL amyloidosis is challenging for clinicians. Substantial delays between onset of symptoms and diagnosis are common, and result in poorer outcomes, particularly in patients with cardiac AL amyloidosis and others who develop advanced organ dysfunction. With the need to identify AL amyloidosis as early as possible, it is important for health-care practitioners, including advanced practice clinicians and nurses, to be aware of the hallmark presenting signs and symptoms, as well as the latest practice for evaluation and diagnosis. Increased awareness of signs and symptoms associated with AL amyloidosis, particularly relating to the most frequently involved organs, the heart and kidneys, represents an opportunity for achieving earlier diagnosis. Here we review these issues in AL amyloidosis, summarize the key presenting symptoms that clinicians need to be alert to, and discuss the latest diagnostic tests, with the aim of expediting patient identification and diagnosis with the goal of improving patient outcomes.