Immunoglobulin Light-chain Amyloidosis

A nonproliferative disorder of PLASMA CELLS characterized by excessive production and misfolding of IMMUNOGLOBULIN LIGHT CHAINS that form insoluble amyloid fibrils (see AMYLOID DEPOSITS) in various tissues. Clinical features include LIVER FAILURE; MULTIPLE MYELOMA; NEPHROTIC SYNDROME; RESTRICTIVE CARDIOMYOPATHY, and neuropathies.

Also Known As:

AL Amyloidosis; Amyloidosis, Immunoglobulin Light-chain; Amyloidosis, Primary; Primary Amyloidosis; Primary Systemic Amyloidosis; AL Amyloidoses; Amyloidoses, Primary; Amyloidoses, Primary Systemic; Amyloidosis, Immunoglobulin Light chain; Amyloidosis, Primary Systemic; Immunoglobulin Light chain Amyloidosis; Immunoglobulin Light-chain Amyloidoses; Primary Amyloidoses; Primary Systemic Amyloidoses; Systemic Amyloidoses, Primary; Systemic Amyloidosis, Primary; Monoclonal Immunoglobulin Deposition Disease

Networked: 2344 relevant articles (179 outcomes, 229 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Multiple Myeloma
2.	Amyloidosis
3.	Paraproteinemias (Monoclonal Gammopathy)
4.	Amyloid Plaque
5.	Heart Failure

Experts

1.	Gertz, Morie A: 139 articles (07/2022 - 03/2002)
2.	Dispenzieri, Angela: 135 articles (01/2022 - 03/2002)
3.	Merlini, Giampaolo: 106 articles (12/2022 - 03/2002)
4.	Sanchorawala, Vaishali: 104 articles (12/2022 - 06/2002)
5.	Palladini, Giovanni: 86 articles (12/2022 - 03/2002)
6.	Lacy, Martha Q: 86 articles (01/2022 - 03/2002)
7.	Leung, Nelson: 74 articles (01/2022 - 01/2005)
8.	Dingli, David: 64 articles (01/2022 - 10/2009)
9.	Hawkins, Philip N: 64 articles (01/2022 - 05/2003)
10.	Kyle, Robert A: 63 articles (01/2022 - 02/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Immunoglobulin Light-chain Amyloidosis:

1.	Melphalan (Alkeran)FDA LinkGeneric 05/01/2006 - "The most effective treatment for systemic AL amyloidosis is risk-adapted melphalan with peripheral blood stem cell transplant (SCT). " 09/01/2010 - "The diagnosis of primary amyloidosis or AL was confirmed and the treatment proposed consisted of high-dose melphalan as conditioning regimen before autologous stem cell transplant, which determined complete remission of the disease, along with the disappearance of clinical signs and absence of the monoclonal component." 03/01/2014 - "Safety and efficacy of high-dose melphalan and auto-SCT in patients with AL amyloidosis and cardiac involvement." 01/01/2010 - "Aggressive treatment with high-dose i.v. melphalan followed by auto-SCT (HDM/SCT) is effective in inducing hematological and clinical remissions, and in extending survival in AL amyloidosis. " 11/01/2013 - "Modified high-dose melphalan and autologous SCT for AL amyloidosis or high-risk myeloma: analysis of SWOG trial S0115."
2.	Bortezomib (Velcade)FDA Link 07/01/2016 - "In the era of bortezomib treatment, the prognosis of AL amyloidosis has been markedly improved and may also result in better post-transplant outcomes. " 01/01/2021 - "Thus, we conclude that pre-transplant bortezomib-based induction was associated with improved relapse/progression and PFS in AL amyloidosis. " 02/01/2008 - "Bortezomib shows promise in the treatment of systemic AL amyloidosis." 02/01/2008 - "Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal disease." 04/01/2021 - "This retrospective case-matched study compares ASCT to bortezomib for the treatment of patients with AL amyloidosis. "
3.	Dexamethasone (Maxidex)FDA LinkGeneric 06/01/1999 - "Phase II trial of high-dose dexamethasone for previously treated immunoglobulin light-chain amyloidosis." 03/01/2010 - "At present, treatment regimes with high-dose dexamethasone are recommended for patients with primary systemic AL-amyloidosis. " 06/01/2001 - "High-dose dexamethasone (HD-Dex) has been reported to benefit AL amyloidosis patients with varying response rates. " 06/01/2001 - "A modified high-dose dexamethasone regimen for primary systemic (AL) amyloidosis." 08/10/2004 - "[Case of Al amyloidosis with severe orthostatic hypotension successfully treated by high-dose dexamethasone]."
4.	Amyloid (Amyloid Fibrils)IBA 04/01/2018 - "The achievement of complete remission of AL-amyloidosis is a precondition for long-term survival, since when not achieved, amyloid deposition also arises in the transplanted heart. " 09/01/2003 - "Relapse occurred after 4 years of complete remission, and the second renal biopsy revealed amyloid light-chain (AL)-amyloidosis. " 12/01/2023 - "REGISTRATION: INPLASY2022100096KEY MESSAGESThis scoping review details the characteristics and results of the recently published studies on diagnosing Amyloid light chain (AL) Amyloidosis in China.Biopsy" 10/24/2021 - "In this study, the impact of lowering the eGFR value on the usefulness of these parameters in the prognosis and diagnosis of the presence of amyloid in the myocardium was assessed in a group of 71 patients with newly diagnosed primary amyloidosis. " 01/01/2021 - "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. "
5.	daratumumabIBA 11/01/2020 - "This study demonstrates that daratumumab is highly effective in the treatment of previously treated AL amyloidosis, and a significant proportion of patients can achieve deep hematologic responses, as well as improvements in organ function." 01/01/2021 - "Daratumumab proved to be highly effective in newly diagnosed AL amyloidosis with excellent hematologic and organ response rates, a remarkable safety profile, and good tolerability even in patients with advanced stage of disease." 01/01/2020 - "Daratumumab is highly effective in heavily pretreated patients with relapsed/refractory AL amyloidosis and high bone marrow plasma cell burden. " 01/01/2019 - "Daratumumab proves safe and highly effective in AL amyloidosis." 09/01/2020 - "Rapid response to single agent daratumumab is associated with improved progression-free survival in relapsed/refractory AL amyloidosis."
6.	Immunoglobulin Light Chains (Immunoglobulin Light Chain)IBA 01/01/2017 - "The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL) amyloidosis. " 01/01/2019 - "In immunoglobulin light-chain (AL) amyloidosis, the depth of hematologic response to treatment is associated with improved survival and organ responses. " 12/01/2018 - "Although survival has improved in recent decades, the short-term prognosis of patients with immunoglobulin light chain (AL) amyloidosis remains grim. " 01/01/2021 - "Objective: This study aimed to better understand the clinical, electrophysiological, pathological features and prognosis of peripheral nerve involvements in primary immunoglobulin light-chain (AL) amyloidosis. " 09/01/2016 - "Our study examines depression and anxiety in patients with immunoglobulin light chain (AL) amyloidosis, and determines the associations between the mental health problems and patient characteristics (age, gender, race, marital status, alcohol consumption, smoking status and cardiac involvement). "
7.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2022 - "the addition of cyclophosphamide to VD was not associated with improved outcomes of patients with AL amyloidosis in this retrospective study." 02/01/2006 - "Six patients aged between 43 and 59 years were diagnosed with AL-amyloidosis and had stem cells mobilized with either recombinant human granulocyte colony-stimulating factor (rhG-CSF) alone (n = 2) or cyclophosphamide (2-4 g/m(2)) and rhG-CSF (n = 4). " 09/01/2014 - "Efficacy of bortezomib, cyclophosphamide and dexamethasone in treatment-naïve patients with high-risk cardiac AL amyloidosis (Mayo Clinic stage III)." 03/01/2013 - "The combination of cyclophosphamide, lenalidomide and dexamethasone is an effective treatment for relapsed/refractory AL amyloidosis, and good quality hematologic response should be the aim of treatment in this setting. " 01/01/2017 - "In conclusion, bortezomib even with low doses of dexamethasone is effective for the treatment of AL amyloidosis; higher doses of dexamethasone and addition of cyclophosphamide do not seem to have a profound effect on efficacy and survival."
8.	Lenalidomide (CC 5013)FDA Link 01/15/2007 - "Findings from this trial indicate that lenalidomide can be effective in treating AL amyloidosis." 07/01/2014 - "Here, we report on a study of patients with AL amyloidosis on dialysis treated with lenalidomide at a dose of 10 mg orally three times a week. " 03/01/2011 - "The objective of this study is to characterize alterations in kidney function among patients with AL amyloidosis undergoing treatment with lenalidomide. " 12/01/2018 - "We conclude that although lenalidomide is a major salvage option for patients with relapsed/refractory AL amyloidosis, its toxicity in patients with AL amyloidosis is significant and doses should be adjusted for optimal tolerability." 12/01/2018 - "We retrospectively evaluated 55 consecutive patients who received at least one dose of lenalidomide for relapsed/refractory AL amyloidosis. "
9.	Biomarkers (Surrogate Marker)IBA 01/01/2017 - "The topics include the limitations of established biomarkers in risk assessment and response evaluation, the introduction of a new biomarker, the comparison of different treatment sequences and the efficacy of a multiple drug regimen in light-chain (AL) amyloidosis." 12/20/2012 - "This study identifies and validates new criteria for response to first-line treatment in AL amyloidosis, based on their association with survival in large patient populations, and offers surrogate end points for clinical trials." 10/01/2021 - "The primary aim of this study was to determine interobserver reproducibility and intraobserver repeatability, defined per the recommendations of the Quantitative Imaging Biomarker Alliance technical performance group, of PET 18F-florbetapir retention index (RI) in patients with cardiac AL amyloidosis. " 01/01/2020 - "MRD assessment should be explored as a surrogate endpoint in clinical trials and MRD risk-adapted trials may help optimize treatment in AL amyloidosis." 01/01/2018 - "The aim of this study was to evaluate the prognostic implication of baseline GLS and the added value of GLS beyond circulating cardiac biomarkers for risk stratification in patients with AL amyloidosis undergoing HCT. "
10.	Thalidomide (Thalomid)FDA Link 10/26/2006 - "One patient, diagnosed as AL amyloidosis with cardiac involvement, was treated with thalidomide and showed remarkable improvement in functional capacity, cardiac function, and various laboratory parameters without any significant adverse effect and, therefore, we report the case with the review of literatures." 10/26/2006 - "An intriguing case of primary amyloidosis with cardiac involvement: symptomatic and echocardiographic improvement with thalidomide treatment." 03/01/2003 - "Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis." 12/01/2003 - "Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis." 03/01/2003 - "Thus, while thalidomide has activity in AL amyloidosis, it also has significant toxicity in this patient population."

Therapies and Procedures

1.	Therapeutics 04/01/2018 - "No specific therapy was needed for the third patient, as immunosuppressive therapy following the heart transplant containing prednison led to complete remission of AL-amyloidosis. " 11/01/2019 - "Survival for patients with systemic AL amyloidosis has improved for patients at all stages of disease in the present era of rapid advancements in light chain-reducing therapies. " 12/01/2018 - "Of note, hematologic and organ responses in elderly patients with AL amyloidosis do translate in better outcome, advocating the importance of treating these patients and striving for a rapid response to therapy also in this challenging clinical setting." 01/01/2018 - "Patients with AL amyloidosis who need second-line therapy after response to up-front treatment generally have a good outcome. " 12/08/2017 - "The future of AL amyloidosis treatment is combination therapy and will require an innovative collaborative model for a rapid translation from bench to bedside with the ultimate aim of achieving a cure for this complex disease."
2.	Stem Cell Transplantation 12/01/2020 - "Stem cell transplantation was one of the first proven effective regimens for the management of immunoglobulin light-chain amyloidosis. " 01/01/2016 - "Autologous stem cell transplantation (SCT) is a common management strategy for select patients with immunoglobulin light-chain amyloidosis, but no trials have documented improved overall survival. " 12/01/1999 - "Autologous stem cell transplantation (ASCT) is a new treatment option in patients with systemic AL amyloidosis (AL). " 09/01/2010 - "AL amyloidosis in a young adult: remission with autologous stem cell transplantation." 01/01/2023 - "This study was conducted to better understand the characteristics of ES following autologous stem cell transplantation (ASCT) in AL amyloidosis with renal involvement. "
3.	Drug Therapy (Chemotherapy) 01/01/2001 - "It was found that high-dose chemotherapy with autologous transpantation of haematopoietic cells is at present the most effective treatment for selected patients with not advanced forms of primary systemic AL-amyloidosis. " 04/01/2018 - "Sometimes one high-dose chemotherapy is sufficient, while at other times multiple treatment lines are needed to reach complete remission of AL-amyloidosis.Key" 11/01/2017 - "Our results show that CAD chemotherapy is very effective in PBSC mobilization and has a tolerable toxicity profile in AL amyloidosis patients. " 03/24/2015 - "Although developments in chemotherapy have greatly improved the outcomes in AL amyloidosis, the prognosis of patients with severe cardiac involvement remains very poor. " 01/01/2017 - "A good clonal response to chemotherapy in AL amyloidosis is associated with improved quality of life and function at 1 year."
4.	Peripheral Blood Stem Cell Transplantation (Peripheral Stem Cell Transplantation) 06/01/2006 - "[White blood cell lysis syndrome after autologous peripheral blood stem cell transplantation in the treatment of renal AL amyloidosis. " 03/01/2005 - "Predictive factors for hematopoietic engraftment after autologous peripheral blood stem cell transplantation for AL amyloidosis." 05/15/2004 - "Superior survival in primary systemic amyloidosis patients undergoing peripheral blood stem cell transplantation: a case-control study." 08/01/1998 - "Gastrointestinal perforation early after peripheral blood stem cell transplantation for AL amyloidosis." 08/01/2008 - "Between 1994 and 2005, 11 patients underwent sequential orthotopic heart transplantation (HT) followed by autologous peripheral blood stem cell transplantation (SCT) for treatment of AL amyloidosis. "
5.	Heart Transplantation (Grafting, Heart) 02/01/2013 - "The woman is in a complete AL amyloidosis remission seven months after the heart transplantation. " 02/01/2013 - "Scheduling heart transplantation before the treatment of AL amyloidosis will significantly improve the patients overall condition and enable them to undergo the intensive AL amyloidosis treatment with the hope that a long-term complete remission may be achieved. " 11/01/2022 - "In systemic light-chain (AL) amyloidosis, cardiac involvement is a major determinant of survival; however, cardiac response is limited even after systemic treatment in a majority of patients, and some require heart transplantation. " 04/01/2018 - "[Heart transplantation and follow-up treatment with AL-amyloidosis in 5 patients]." 01/01/2018 - "We describe our experience from the two first patients with AL amyloidosis treated with heart transplantation and subsequent ASCT at Sahlgrenska University Hospital."