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

作者: Yi L. Hwa, DNP , Teresa Fogaren, MSN, AGNP-C , Allison Sams, AGPCNP-BC , Douglas V. Faller, MD, PhD , Dawn M. Stull, PharmD, BCOP

DOI: 10.6004/JADPRO.2019.10.5.5

关键词: Immunoglobulin Light-chain AmyloidosisAL amyloidosisPatient identificationOrgan dysfunctionSigns and symptomsMedicineAmyloidosisIn patientAmyloid fibrilIntensive care medicine

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

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