scholarly journals Primary treatment of light-chain amyloidosis with bortezomib, lenalidomide, and dexamethasone

2019 ◽  
Vol 3 (20) ◽  
pp. 3002-3009 ◽  
Author(s):  
Efstathios Kastritis ◽  
Ioanna Dialoupi ◽  
Maria Gavriatopoulou ◽  
Maria Roussou ◽  
Nikolaos Kanellias ◽  
...  
Keyword(s):  
Light Chain ◽  
Low Dose ◽  
Advanced Disease ◽  
Primary Treatment ◽  
Al Amyloidosis ◽  
Active Therapy ◽  
Light Chain Amyloidosis ◽  
Nonhematologic Toxicity ◽  
Key Points

Key Points Bortezomib with dexamethasone and low-dose lenalidomide is an active therapy for previously untreated patients with AL amyloidosis. VRD can induce MRD-negative responses, but nonhematologic toxicity may be significant in patients with advanced disease.

scholarly journals Patients with light-chain amyloidosis and low free light-chain burden have distinct clinical features and outcome

Blood ◽  
2017 ◽  
Vol 130 (5) ◽  
pp. 625-631 ◽  
Author(s):  
Paolo Milani ◽  
Marco Basset ◽  
Francesca Russo ◽  
Andrea Foli ◽  
Giampaolo Merlini ◽  
...  
Keyword(s):  
Clinical Features ◽  
Light Chain ◽  
Free Light Chain ◽  
Al Amyloidosis ◽  
Renal Survival ◽  
Light Chain Amyloidosis ◽  
Hematologic Response ◽  
Heart Involvement ◽  
Key Points

Key PointsPatients with AL amyloidosis and low dFLC burden (<50 mg/L) have less severe heart involvement and better survival. These patients are evaluable for hematologic response with adapted criteria predicting improvement of overall and renal survival.

A phase 1/2 study of lenalidomide with low-dose oral cyclophosphamide and low-dose dexamethasone (RdC) in AL amyloidosis

Blood ◽  
2012 ◽  
Vol 119 (23) ◽  
pp. 5384-5390 ◽  
Author(s):  
Efstathios Kastritis ◽  
Evangelos Terpos ◽  
Maria Roussou ◽  
Maria Gavriatopoulou ◽  
Constantinos Pamboukas ◽  
...  
Keyword(s):  
Light Chain ◽  
Low Dose ◽  
Phase 1 ◽  
Al Amyloidosis ◽  
Oral Cyclophosphamide ◽  
Phase 2 ◽  
Light Chain Amyloidosis ◽  
Intention To Treat ◽  
Oral Regimen ◽  
Dose Oral

Abstract In this phase 1/2 study, we explored the feasibility and activity of an oral regimen of lenalidomide with low-dose dexamethasone and low-dose oral cyclophosphamide (RdC) in patients with primary systemic light chain amyloidosis. RdC was given for up to 12 cycles in prespecified cohorts at escalated doses: 13 patients were treated in phase 1 and 24 in phase 2; 65% were previously untreated, and most had renal and/or cardiac involvement and elevated cardiac biomarkers. Lenalidomide 15 mg/d and cyclophosphamide 100 mg/d were further evaluated in phase 2. On intention to treat, 20 (55%) patients achieved a hematologic response, including 3 (8%) complete remissions. Hematologic responses were seen at all dose levels and in 4 of 5 patients who had received bortezomib previously. An organ response was recorded in 22% of patients on intention-to-treat and in 40% of patients who survived at least 6 months. The median time to progression was 10 months and the 2-year survival was 41%. Fatigue, nonneutropenic infections, and rash were the most common toxicities. The results of the present study show that RdC is an oral regimen with activity in primary systemic light chain amyloidosis and may be an additional treatment option, especially for patients with preserved organ function or for patients who cannot receive or who relapse after bortezomib. This study is registered at www.clinicaltrials.gov as NCT00981708.

scholarly journals Phenotypic, transcriptomic, and genomic features of clonal plasma cells in light-chain amyloidosis

Blood ◽  
2016 ◽  
Vol 127 (24) ◽  
pp. 3035-3039 ◽  
Author(s):  
Bruno Paiva ◽  
Joaquin Martinez-Lopez ◽  
Luis A. Corchete ◽  
Beatriz Sanchez-Vega ◽  
Inmaculada Rapado ◽  
...  
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Al Amyloidosis ◽  
Genomic Features ◽  
Light Chain Amyloidosis ◽  
Key Points

Key Points Clonal PCs in AL have similar phenotypic and CNA profiles as those in MM, but their transcriptome is similar to that of normal PCs. First-ever WES in AL amyloidosis reveals potential lack of a unifying mutation.

scholarly journals Growth differentiation factor-15 is a new biomarker for survival and renal outcomes in light chain amyloidosis

Blood ◽  
2018 ◽  
Vol 131 (14) ◽  
pp. 1568-1575 ◽  
Author(s):  
Efstathios Kastritis ◽  
Ioannis Papassotiriou ◽  
Giampaolo Merlini ◽  
Paolo Milani ◽  
Evangelos Terpos ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Light Chain ◽  
Al Amyloidosis ◽  
Growth Differentiation Factor 15 ◽  
Renal Outcomes ◽  
Light Chain Amyloidosis ◽  
Differentiation Factor ◽  
Key Points

Key Points GDF-15 level is a new prognostic factor for survival in AL amyloidosis, and its reduction after therapy is associated with better outcome. GDF-15 level is probably the strongest predictor for renal outcomes in patients with AL amyloidosis.

scholarly journals Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil
Keyword(s):  
Differential Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Diagnosis And Treatment ◽  
Diagnostic Approach ◽  
Al Amyloidosis ◽  
Delay In Diagnosis ◽  
Light Chain Amyloidosis ◽  
Novel Approaches

AbstractCardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

scholarly journals Light chain type predicts organ involvement and survival in AL amyloidosis patients receiving stem cell transplantation

2018 ◽  
Vol 2 (7) ◽  
pp. 769-776 ◽  
Author(s):  
M Hasib Sidiqi ◽  
Mohammed A. Aljama ◽  
Eli Muchtar ◽  
Francis K. Buadi ◽  
Rahma Warsame ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Light Chain ◽  
Organ Involvement ◽  
Al Amyloidosis ◽  
Light Chain Type ◽  
Key Points ◽  
Λ Light Chain ◽  
Chain Type

Key Points λ Light chain AL amyloidosis is associated with a shorter PFS and OS compared with κ. Light chain type predicts likelihood of organ involvement in AL amyloidosis.

Primary Treatment of Light Chain (AL) Amyloidosis With Bortezomib, Lenalidomide and Dexamethasone (VRD) or with Bortezomib, Cyclophosphamide and Dexamethasone (VCD/CyBorD): efficacy and toxicity

2019 ◽  
Vol 19 (10) ◽  
pp. e320-e321
Author(s):  
Efstathios Kastritis ◽  
Maria Gavriatopoulou ◽  
Despina Fotiou ◽  
Nikolaos Kanellias ◽  
Magdalini Migkou ◽  
...  
Keyword(s):  
Light Chain ◽  
Primary Treatment ◽  
Al Amyloidosis

scholarly journals Hereditary systemic immunoglobulin light-chain amyloidosis

Blood ◽  
2015 ◽  
Vol 125 (21) ◽  
pp. 3281-3286 ◽  
Author(s):  
Merrill D. Benson ◽  
Juris J. Liepnieks ◽  
Barbara Kluve-Beckerman
Keyword(s):  
Light Chain ◽  
Plasma Cell Dyscrasia ◽  
Al Amyloidosis ◽  
Constant Region ◽  
Immunoglobulin Light Chain ◽  
Region Gene ◽  
Dna Analyses ◽  
Key Points ◽  
Constant Region Gene ◽  
Immunoglobulin Light Chain Amyloidosis

Key Points Protein and DNA analyses reveal that mutation in the immunoglobulin κ light-chain constant region gene may cause hereditary amyloidosis. Sequencing of immunoglobulin light-chain constant region genes is indicated for patients with AL amyloidosis and no evidence of a plasma cell dyscrasia.

scholarly journals Electrocardiogram Characteristics and Prognostic Value in Light-Chain Amyloidosis: A Comparison With Cardiac Magnetic Resonance Imaging

2021 ◽  
Vol 8 ◽  
Author(s):  
Xinli Guo ◽  
Zhian Chen ◽  
Ke Wan ◽  
Rizhen Song ◽  
Tingjie Yang ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Light Chain ◽  
Prognostic Value ◽  
Cox Regression ◽  
Extracellular Volume ◽  
Al Amyloidosis ◽  
Amyloid Burden ◽  
Light Chain Amyloidosis ◽  
Fragmented Qrs

Background: An electrocardiogram (ECG) is a simple and cheap non-invasive tool that shows various abnormalities and has prognostic value for patients with light-chain amyloidosis (AL). The present study aimed to explore the association between ECG characteristics and cardiac magnetic resonance (CMR)-detected amyloid burden and to investigate the prognostic value of ECG in AL amyloidosis.Methods: We prospectively enrolled 99 patients with AL amyloidosis (56 male patients; median age, 58 y). Detailed clinical information, 12-lead ECG, and CMR data were collected. All patients were followed up longitudinally, and the endpoint was all-cause mortality. ECG characteristics were analyzed and correlated with the degree of late gadolinium enhancement (LGE) and extracellular volume (ECV) by T1 mapping on CMR. The prognostic value of ECG characteristics was analyzed using Kaplan–Meier survival analysis and multivariate Cox regression.Results: During a median follow-up period of 33 months, 69 of the 99 patients died. Fragmented Q wave-R wave-S wave (QRS), pathological Q waves, the Sokolow index, QRS duration, and voltages were significantly associated with the extent of LGE, native T1, and ECV by CMR (p &lt; 0.05). Fragmented QRS and Sokolow index showed independent prognostic value in AL amyloidosis (p = 0.001; p = 0.026, respectively). Fragmented QRS remained independent after adjusting for clinical values (hazard ratio: 2.034; 95% confidence interval: 1.148–3.603; p = 0.015). However, no ECG characteristics were independent predictors for prognosis in AL amyloidosis when LGE and ECV were included in the multivariate analysis.Conclusion: ECG abnormalities showed significant association with CMR indicators of amyloid burden. Fragmented QRS has an independent prognostic value in AL amyloidosis and could be used as an alternative marker when CMR is not available.

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