Systemic Light Chain Research Articles

A Medical Chart Review of Healthcare Costs in Patients with Systemic Amyloid Light Chain Amyloidosis By Mayo 2012 Stage in the United States

A Medical Chart Review of Healthcare Costs in Patients with Systemic Amyloid Light Chain Amyloidosis By Mayo 2012 Stage in the United States

Autologous Stem Cell Transplantation (ASCT) for Light Chain (AL) Amyloidosis: A Single-Center Report

Context: Since the 1990s, high-dose intravenous melphalan followed by ASCT has been a cornerstone in treating AL amyloidosis. In recent years, there has been an increase in the therapeutic arsenal for this condition; however, ASCT, based on melphalan, continues to be recommended due to its high response rates and prolonged duration, leading to long periods of remission, including better overall survival (OS). Objective/Methods: This is an observation retrospective case-series study encompasses a consecutive cohort of patients submitted to ASCT to treat systemic light chain amyloidosis. We reviewed files of patients who underwent ASCT between 2016 and 2024 at Beneficência Portuguesa de São Paulo to assess the procedure's safety. Our analysis focused on patient baseline characteristics, progression-free survival (PFS), intensive care unit (ICU) admission rates, and overall survival (OS). The present study received approval from the local ethics committee and adhered to the principles outlined in the Declaration of Helsinki and the Guidelines for Good Clinical Practice. Results: Twenty-seven patients met the criteria for this retrospective study. The median age was 60 years (95% CI, 38-70) and time from diagnosis to aHSCT was 232 days (IQR 170-387). Seventeen patients (63%) had an ECOG score of 0, and 11 (40%) had a Mayo Cardiac score of 1. Cardiac involvement was present in 11 patients and renal involvement in 23. Among the cohort, 81% (95% CI, 61-92) received ASCT as part of first-line therapy, after an induction, while five patients (18%) received no therapy prior to ASCT. Twelve patients (44%) received a CyBorDex regimen, and 9 (33%) received a Dara-based quadruplet regimen. The most used conditioning regimen was melphalan 200 mg/m² (55.6%), with the remaining patients receiving 140 mg/m².The vast majority (24/27) received non-cryopreserved ASCT. During hospitalization, 11 patients experienced congestive heart failure decompensation (four profile ‘B’ and seven profile ‘C‘), with a higher risk observed in patients with pre-existing cardiac dysfunction (OR=5.2; 95% CI, 0.92-47; P=0.06). None of the patients were on renal replacement therapy at baseline; however, four required hemodialysis during hospital follow-up. There were no in-hospital deaths in this cohort, although 33% of patients were admitted to the ICU within 30 days of follow-up (95% CI, 16-51). The cohort's median follow up was 749 days, the overall survival (OS) at 2 years was 88% (CI95% 73-100) and progression free survival (PFS) at 2 years was 89% (CI95% 76-100),with no early deaths (within 100 days of ASCT). Conclusion: The present analysis reiterates the critical importance of of meticulous patient selection for selection for ASCT, given the potential for serious complications. Therefore, a transplant center's experience plays pivotal role in ensuring optimal outcomes. In our cohort, the majority of patients survived, with no transplant-related deaths occurring within the first 100 days. Real-world studies are crucial in medical research as they provide insights into the effectiveness, safety, and practical application of treatments and interventions in diverse, everyday clinical settings, improving patient outcomes by providing a more comprehensive understanding of treatment efficacy and safety in real-world conditions.

Differential Diagnosis of Multiple Myeloma and Systemic Light Chain Amyloidosis Using Plasma Cell Morphology Based on Deep Learning

Differential Diagnosis of Multiple Myeloma and Systemic Light Chain Amyloidosis Using Plasma Cell Morphology Based on Deep Learning

Gain or Amplification of 1q21 in Systemic Light Chain Amyloidosis Is Associated with Advanced Mayo Stage, Plasma Cell Disease and Worse Overall Survival

Gain or Amplification of 1q21 in Systemic Light Chain Amyloidosis Is Associated with Advanced Mayo Stage, Plasma Cell Disease and Worse Overall Survival

Therapeutic Efficacy Analysis of 16 Patients with Primary Systemic Light Chain Amyloidosis Predominantly Exhibiting Advanced Cardiac Amyloidosis

Therapeutic Efficacy Analysis of 16 Patients with Primary Systemic Light Chain Amyloidosis Predominantly Exhibiting Advanced Cardiac Amyloidosis

Why is Dimeric 3D Domain Swapping in Antibody Light Chains Missing from the Solution? Atomistic Insights Mechanisms.

Misfolding of antibody light chains can lead to systemic light chain amyloidosis, which is associated with misfolding and aggregation. The antibody light chain may engage in 3D domain swapping within the variable region (#4VL) through hydrogen bonding (HB) interactions, potentially forming the tetramer, as revealed in solution and crystal structures. However, the 3D-domain swapping (3D-DS) dimers could not be detected experimentally. This study investigates the absence of 3D-DS using computational approaches, focusing on structural dynamics, solvation effects, and stability relevant to the loss of 3D-DS. Microscale molecular dynamics simulations of #4VL and 3D-DS confirm that native HB interactions are essential to maintain β-sheet structures in both #4VL and 3D-DS. A flickering native HB interaction in the 3D-DS system, caused by repulsive interaction with water molecules in the hydrophobic region, leads to intramolecular breathing motions and oligomerization in another 3D-DS. Structural dynamics of the 3D-DS dimer in long-run simulations were analyzed using the newly developed integrated solvation-based principal component analysis (3D-RISM/PCA) and density-based spatial clustering of applications with noise, confirm that if the 3D-DS cannot form the tetramer within the breathing motion process, the 3D-DS will collapse. This finding provides insights into why the 3D-DS dimer is missing from the solution and can be used to design and develop 3D-DS in other antibodies.

Changes in the clinical features, treatments, and outcomes of patients with systemic light chain (AL) amyloidosis in Western China, 2010-2022: A multicenter, retrospective, real-world study.

Changes in the clinical features, treatments, and outcomes of patients with systemic light chain (AL) amyloidosis in Western China, 2010-2022: A multicenter, retrospective, real-world study.

Dexamethasone for the Treatment of Cardiac Al Amyloidosis: A Case Report and Literature Review

Background: Systemic immunoglobulin light chain (AL) amyloidosis is a condition characterised by the conversion of light chains (LCs) from their soluble states into highly organised fibrillar aggregates that deposit in tissues, resulting in progressive organ damage and dysfunction. While previously considered incurable, treatment strategies are emerging for cardiac amyloidosis, underscoring the importance of early diagnosis. Case Summary: We present the case of a 69-year-old male patient who was admitted with acute onset of dyspnea and diagnosed with heart failure. The diagnosis of cardiac amyloidosis was made on the basis of a discrepancy between the results of the electrocardiogram and the echocardiogram, a conclusion that was further substantiated by the use of scintigraphy and myelogram. Given the poor left ventricular fraction, the standard chemotherapy regimen was replaced with corticosteroid therapy, which led to a significant improvement within the first 48 hours, unfortunately reversible with rapid clinical deterioration followed by sudden death within a week. Conclusion: Our aim is to highlight that early administration of dexamethasone is associated with significant clinical improvement but a high mortality rate in patients with AL cardiac amyloidosis.

Redefining Cardiac Involvement and Targets of Treatment in Systemic Immunoglobulin AL Amyloidosis

Cardiac amyloid infiltration is the key determinant of survival in systemic light-chain (AL) amyloidosis. Current guidelines recommend early switching therapy in patients with a nonoptimal or suboptimal response regardless of the extent of cardiac amyloid infiltration. To assess the differences between serum biomarkers, echocardiography, and cardiovascular magnetic resonance (CMR) with extracellular volume (ECV) mapping in characterizing cardiac amyloid, the independent prognostic role of these approaches, and the role of ECV mapping to guide treatment strategies. Consecutive patients newly diagnosed with systemic AL amyloidosis (2015-2021) underwent echocardiography, cardiac biomarkers, and CMR with ECV mapping at diagnosis. Data were analyzed from January to June 2024. The primary outcomes of the study were all-cause mortality and hematological response as defined according to validated criteria: no response (NR), partial response (PR), very good partial response (VGPR), and complete response (CR). Secondary outcomes were the depth and speed of hematological response and overall survival according to ECV. Of 560 patients with AL amyloidosis, the median (IQR) age was 68 years (59-74 years); 346 patients were male (61.8%) and 214 female (38.2%). Over a median (IQR) 40.5 months 9-58 months), ECV was independently associated with mortality. In the landmark analysis at 1 month, long-term survival was independent of the achieved hematological response in ECV less than 0.30% and ECV of 0.31% to 0.40%, while it was dependent on the depth of the hematological response in ECV greater than 0.40%. In the landmark analysis at 6 months, survival was independent of the achieved hematological response in ECV less than 0.30% and dependent on achieving at least PR in ECV of 0.31% to 0.40%. Survival was dependent on achieving CR in ECV of 0.41% to 0.50% and ECV greater than 0.50%. Achieving a deep hematological response at 1 month was associated with better survival compared with 6 months in patients with ECV greater than 0.40% but not with ECV less than 0.40%. This study found that ECV mapping, in systemic AL amyloidosis, is an independent predictor of prognosis, can help define the hematological response associated with better long-term outcomes for each patient and potentially inform treatment strategies.

Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis.

To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis. Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement. Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4kPa vs. 8.1kPa; p = 0.001) and SAP scintigraphy (median 20.9kPa vs. 6.2kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard. Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.

Helical superstructures between amyloid and collagen in cardiac fibrils from a patient with AL amyloidosis

Systemic light chain (LC) amyloidosis (AL) is a disease where organs are damaged by an overload of a misfolded patient-specific antibody-derived LC, secreted by an abnormal B cell clone. The high LC concentration in the blood leads to amyloid deposition at organ sites. Indeed, cryogenic electron microscopy (cryo-EM) has revealed unique amyloid folds for heart-derived fibrils taken from different patients. Here, we present the cryo-EM structure of heart-derived AL amyloid (AL59) from another patient with severe cardiac involvement. The double-layered structure displays a u-shaped core that is closed by a β-arc lid and extended by a straight tail. Noteworthy, the fibril harbours an extended constant domain fragment, thus ruling out the variable domain as sole amyloid building block. Surprisingly, the fibrils were abundantly concatenated with a proteinaceous polymer, here identified as collagen VI (COLVI) by immuno-electron microscopy (IEM) and mass-spectrometry. Cryogenic electron tomography (cryo-ET) showed how COLVI wraps around the amyloid forming a helical superstructure, likely stabilizing and protecting the fibrils from clearance. Thus, here we report structural evidence of interactions between amyloid and collagen, potentially signifying a distinct pathophysiological mechanism of amyloid deposits.

Prognostic Value of Left Ventricular 18F-Florbetapir Uptake in Systemic Light-Chain Amyloidosis

BackgroundPositron emission tomography/computed tomography (PET/CT) with 18F-florbetapir, a novel amyloid-targeting radiotracer, can quantify left ventricular (LV) amyloid burden in systemic light-chain (AL) amyloidosis. However, its prognostic value is not known. ObjectivesThe authors’ aim was to evaluate the prognostic value of LV amyloid burden quantified by 18F-florbetapir PET/CT, and to identify mechanistic pathways mediating its association with outcomes. MethodsA total of 81 participants with newly diagnosed AL amyloidosis underwent 18F-florbetapir PET/CT imaging. Amyloid burden was quantified using 18F-florbetapir LV uptake as percent injected dose. The Mayo stage for AL amyloidosis was determined using troponin T, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain levels. Major adverse cardiac events (MACE) were defined as all-cause death, heart failure hospitalization, or cardiac transplantation within 12 months. ResultsAmong participants (median age, 61 years; 57% males), 36% experienced MACE, increasing from 7% to 63% across tertiles of LV amyloid burden (P < 0.001). LV amyloid burden was associated with MACE (HR: 1.46; 95% CI: 1.16-1.83; P = 0.001). However, this association became nonsignificant when adjusted for Mayo stage. In mediation analysis, the association between LV amyloid burden and MACE was mediated by NT-proBNP (P < 0.001), a marker of cardiomyocyte stretch and heart failure, and a component of Mayo stage. ConclusionsIn this first study to link cardiac 18F-florbetapir uptake to subsequent outcomes, LV amyloid burden estimated by percent injected dose predicted MACE in AL amyloidosis. This effect was not independent of Mayo stage and was mediated primarily through NT-proBNP. These findings provide novel insights into the mechanism linking myocardial amyloid deposits to MACE.

Myocardial Characteristics, Cardiac Structure, and Cardiac Function in Systemic Light-Chain Amyloidosis

BackgroundIn systemic light-chain (AL) amyloidosis, cardiac involvement portends poor outcomes. ObjectivesThe authors’ objectives were to detect early myocardial alterations, to analyze longitudinal changes with therapy, and to predict major adverse cardiac events (MACE) in participants with AL amyloidosis using cardiac magnetic resonance imaging (MRI). MethodsRecently diagnosed participants were prospectively enrolled. AL amyloidosis with and without cardiomyopathy (AL-CMP, AL-non-CMP) were defined based on abnormal cardiac biomarkers and wall thickness. MRI was performed at baseline, 6 months in all participants, and 12 months in participants with AL-CMP. MACE were defined as all-cause death, heart failure hospitalization, and cardiac transplantation. Mayo stage was based on troponin T, N-terminal pro–B-type natriuretic peptide, and difference in free light chains. ResultsThis study included 80 participants (median age 62 years, 58% men). Extracellular volume (ECV) was abnormal (>32%) in all participants with AL-CMP and in 47% of those with AL-non-CMP. ECV tended to increase at 6 months (median +2%; AL-CMP P = 0.120; AL-non-CMP P = 0.018) and returned to baseline values at 12 months in participants with AL-CMP. Global longitudinal strain (GLS) improved at 6 months (median −0.6%; P = 0.048) and 12 months (median −1.2%; P < 0.001) in participants with AL-CMP. ECV and GLS were strongly associated with MACE (P < 0.001) and improved the prognostic value when added to Mayo stage (P ≤ 0.002). No participant with ECV ≤32% had MACE, while 74% of those with ECV >48% had MACE. ConclusionsIn patients with systemic AL amyloidosis, ECV detects subclinical myocardial alterations. With therapy, ECV tends to increase at 6 months and returns to values unchanged from baseline at 12 months, whereas GLS improves at 6 and 12 months in participants with AL-CMP. ECV and GLS offer additional prognostic performance over Mayo stage. (Molecular Imaging of Primary Amyloid Cardiomyopathy [MICA]; NCT02641145)

Internalisation of immunoglobulin light chains by cardiomyocytes in AL amyloidosis: what can biopsies tell us?

Background Cardiac involvement in systemic light chain amyloidosis (AL) leads to chronic heart failure and is a major prognosis factor. Severe cellular defects are provoked in cardiac cells by tissue-deposited amyloid fibrils of misfolded free immunoglobulin light chains (LCs) and their prefibrillar oligomeric precursors. Objective Understanding the molecular mechanisms behind cardiac cell cytotoxicity is necessary to progress in therapy and to improve patient management. One key question is how extracellularly deposited molecules exert their toxic action inside cardiac cells. Here we searched for direct evidence of amyloid LC uptake by cardiomyocytes in patient biopsies. Methods We immunolocalized LCs in cardiac biopsies from four AL cardiac amyloidosis patients and analysed histopathological images by high resolution confocal microscopy and 3D image reconstruction. Results We show, for the first time directly in patient tissue, the presence of LCs inside cardiomyocytes, and report their proximity to nuclei and to caveolin-3-rich areas. Our observations point to macropinocytosis as a probable mechanism of LC uptake. Conclusions Internalisation of LCs occurs in patient cardiomyocytes. This event could have important consequences for the pathogenesis of the cardiac disease by enabling interactions between amyloid molecules and cellular organelles inducing specific signalling pathways, and might bring new insight regarding treatment.

Multimodal Imaging of Bilateral Retinal Pigment Epithelial Immunoglobulin Light Chain Deposition in Patients with Systemic Immunoglobulin Light Chain Deposition

ObjectiveTo describe multimodal imaging of peculiar bilateral globular subretinal deposits and acquired serous retinal detachment in patients with systemic immunoglobulin light chain deposition. DesignA retrospective observational case series. ParticipantsWe examined six eyes in three patients (one with multiple myeloma, one with membranous nephropathy, and one with immunoglobulin A nephropathy) at the Eye and ENT Hospital of Fudan University. The patients presented with peculiar globular subretinal deposits along the retinal pigment epithelium (RPE)‒Bruch’s membrane complex and acquired serous retinal detachment. MethodsFundus appearance was documented with multimodal imaging, which included fundus photography, fundus autofluorescence, spectral domain optical coherence tomography (OCT), swept-source OCT (SS-OCT), en-face OCT, and SS-OCT angiography. Additional evaluations included serum protein electrophoreses, positron emission tomography computed tomography, and renal and bone biopsies to assess the primary diseases. Main Outcome MeasuresMultimodal imaging, course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition. ResultsBilateral, multiple, speckled, or patchy RPE changes in the posterior fundus that corresponded to striking multifocal hyperautofluorescence on fundus autofluorescence and lumpy, globular hyperreflective deposits along the RPE‒Bruch’s membrane complex were identified as characteristic features of bilateral RPE light chain deposition. These features may be accompanied by dense light chain deposits in the choriocapillaris and choroid vessels, diffuse choroidal thickening, and “angiographically silent” serous retinal detachment in patients with systemic immunoglobulin light chain deposition. ConclusionsWe have documented the characteristic features, clinical course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition. Appropriate evaluations, including serum protein electrophoresis and hematologic consultation, are recommended to manage patients with this fundus abnormality.

Systemic Light Chain (Kappa Type) Amyloidosis Involving Liver and Bone Marrow, Heart, Lung

Systemic amyloidosis is characterized by the accumulation of insoluble proteins in tissues including heart, kidney, liver and any other organs. Light chain amyloidosis is the most common type of primary amyloidosis. And it is generally considered to be the plasma cell dysfunction. Given its pathogenesis, it may affect any organ system. Thus, clinical presentation is variable and delayed diagnosis is common. Given these diagnostic difficulties, we presented a systemic amyloidosis presented as liver dysfunction.

Timing and co-occurrence of symptoms prior to a diagnosis of light chain (AL) amyloidosis

It is well-established that most patients with systemic light chain (AL) amyloidosis have multi-organ involvement and are often diagnosed after a lag period of increasing symptoms. We leverage electronic health record (EHR) data from the TriNetX research network to describe the incidence, timing, and co-occurrence of precursor conditions of interests in a cohort of AL amyloidosis patients identified between October 2015-December 2020. Nineteen precursor diagnoses of interest representing features of AL amyloidosis were identified using ICD codes up to 36 months prior to AL amyloidosis diagnosis. Among 1,401 patients with at least 36 months of EHR data prior to AL amyloidosis diagnosis, 46% were females, 16% were non-Hispanic Black, and 6% were Hispanic. The median age was 71 (range, 21–91) years. The median number of precursor diagnoses was 5 with dyspnea and fatigue being the most prevalent. The time from the first occurrence of a precursor to AL diagnosis ranged from 3.2 to 21.4 months. Analyses of pairwise co-occurrence of specific diagnoses indicated a high association (Cole’s coefficient >0.6) among the examined precursor diagnoses. These findings provide novel information about the timing and co-occurrence of key precursor conditions and could be used to develop algorithms for early identification of AL amyloidosis.

AL amyloidosis: an overview on diagnosis, staging system, and treatment

Systemic light-chain (AL) amyloidosis is a monoclonal plasma cell disease characterized by the deposition of amyloidogenic monoclonal light-chain fragments in organs, causing their dysfunction. Clinical manifestations could be very aspecific, but the most frequent ones are proteinuria with or without renal failure or heart failure, with the kidney and the heart being the first two involved organs. Histological diagnosis with Congo red staining is the gold standard, but typing the amyloid with immunohistochemistry or mass spectrometry of the Congo red positive tissue is necessary to establish if an AL or ATTR amyloidosis could be diagnosed. Staging AL amyloidosis before treatment could help physicians to prognosticate the disease. Recently, staging systems were set separately for different involved organs, using biomarkers. Autologous stem cell transplant after a daratumumab-based induction treatment is the cornerstone of therapy in younger and fit patients, with the goal of reaching a deep and rapid disease hematological and organ response. Novel therapies, borrowed from a therapeutical model of multiple myeloma, are studied to optimize AL amyloidosis outcomes. In this review, we make an overview of diagnostic procedures, staging system, and therapies of AL amyloidosis.

Minimal residual disease in systemic light chain amyloidosis: a systematic review and meta-analysis

PurposeMinimal residual disease (MRD) is a validated prognostic factor in several hematological malignancies. However, its role in systemic light chain (AL) amyloidosis remains controversial, and this systematic review and meta-analysis aims to fill this gap.MethodsWe searched for relevant studies on Pubmed, Embase, and Cochrane Controlled Register of Trials, nine studies involving 451 patients were included and meta-analyzed. This systematic review has been registered in PROSPERO (CRD42023494169).ResultsOur study found that in the group of patients who achieved very good partial response (VGPR) or better, MRD negativity was correlated with higher cardiac and renal response rates [pooled risk ratio (RR) = 0.74 (95% CI 0.62–0.89), 0.74 (95% CI 0.64–0.87), respectively]. Patients with MRD positivity had a higher hematologic progression rate within two years after MRD detection [pooled RR = 10.31 (95% CI 2.02–52.68)]; and a higher risk of hematologic + organ progression in the first year [pooled RR = 12.57 (95% CI 1.73–91.04)]. Moreover, MRD negativity was correlated with a better progression-free survival (PFS) [pooled hazard ratio (HR) = 0.27 (95% CI 0.17–0.45)]; but it did not significantly improve the overall survival (OS) [pooled HR = 0.34 (95% CI 0.11–1.07)].ConclusionIn AL amyloidosis, our study supports that MRD negativity correlates with higher cardiac or renal response rates and indicates a better PFS in the follow-up. However, the correlation between OS and the status of MRD is not significant.

Development and initial validation of the AL-PROfile patient-reported outcome measure in light chain (AL) amyloidosis.

To evaluate the psychometric properties of the AL-PROfile, a patient-reported outcome measure combining the Patient-Reported Outcomes Measurement Information System (PROMIS)-29, two items from PROMIS Cognitive Function, and select Patient-Reported Outcomes version of the Common Terminology Criteria for Adverse Events (PRO-CTCAE) items. Content validity was assessed through cognitive debriefing interviews of 20 patients who completed the AL-PROfile (Study 1). Study 2 involved 297 participants who completed the AL-PROfile and Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). Reliability (internal consistency and test-retest reliability) and validity (convergent and discriminant validity, known groups validity by stage/organ involvement) were calculated. Study 1 participants found the AL-PROfile straightforward confirming the relevance of the included content. Some felt that certain questions were not related to their amyloidosis experience. Study 2 demonstrated acceptable internal consistency for all domains/items except PROMIS Cognitive Function and acceptable test-retest reliability for all except PROMIS Cognitive Function and PRO-CTCAE nausea. Large correlations were seen for the same domain across measures while correlations for divergent domains within a measure and different domains across different measures were small. The PRO-CTCAE items showed small to medium correlations with each other and with PROMIS and SF-36 domains. Stage was associated with physical function, fatigue, social roles, swelling, and shortness of breath scores. The AL-PROfile has acceptable reliability and validity for use in systemic light chain amyloidosis patients.