Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021

Abstract

Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients presenting with these symptoms. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis. Overlooking the diagnosis of light chain amyloidosis leading to therapy delay is common, and it represents an error of diagnostic consideration. Herein we provide a review of established and investigational treatments for patients with AL amyloidosis and provide algorithms for workup and management of these patients.

Fig. 1. Diagnostic algorithm for patients that are being evaluated for a syndrome compatible with systemic amyloidosis.

ATTR transthyretin amyloidosis, AL immunoglobulin light chain amyloidosis, TTR transthyretin, BMB bone marrow biopsy, SIFE serum immunofixation electrophoresis, UIFE urine immunofixation electrophoresis, LC/MS liquid chromatography-coupled tandem mass spectrometry, PYP (99 m)Tc-pyrophosphate scintigraphy. Figure reproduced from “Two types of amyloid in a single patient: a case series”.

Fig. 2. Current treatment algorithm in light chain amyloidosis.

CybBord cyclophosphamide, bortezomib, and dexamethasone, ASCT autologous stem cell transplant, VGPR very good partial response, Len lenalidomide, Pom pomalidomide, Dex dexamethasone. ^Where available induction with daratumumab plus CyBord is recommended. If unavailable CyBord is induction is recommended. *Daratumumab base treatment is preferred at first relapse if patient is previously unexposed to this agent.