scholarly journals The Occurrence of AL Amyloidosis (Light-Chain Amyloidosis) in Patients with Multiple Myeloma in Lower Silesia Region, Poland

2014 ◽  
Vol 23 (2) ◽  
pp. 235-244
Author(s):  
Lidia Usnarska-Zubkiewicz ◽  
Jadwiga Hołojda ◽  
Michał Jeleń ◽  
Anna Zubkiewicz-Zarębska ◽  
Jakub Dębski ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Al Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Lower Silesia

Daratumumab, pomalidomide, and dexamethasone as a bridging therapy to autologous stem cell transplantation in a case of systemic light-chain amyloidosis with advanced cardiac involvement

2018 ◽  
Vol 25 (4) ◽  
pp. 1021-1025 ◽  
Author(s):  
Justin R Arnall ◽  
Saad Z Usmani ◽  
Hawawu Adamu ◽  
Joseph Mishkin ◽  
Manisha Bhutani
Keyword(s):  
Multiple Myeloma ◽  
Abdominal Pain ◽  
Light Chain ◽  
Cardiac Involvement ◽  
Single Agent ◽  
Al Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Hematologic Disorder ◽  
Complete Hematologic Response ◽  
Paradoxical Worsening

Systemic light-chain (AL) amyloidosis is a rare hematologic disorder where proteins infiltrate tissues leading to organ failure and death. Cardiac involvement, present in ∼70% of patients, determines stage and prognosis of the disease, with advanced involvement having a median survival of six months. The treatment of light-chain amyloidosis is directed at recovering organ function with therapeutic strategies following those of multiple myeloma with plasma cell-directed therapies. The use of single agent daratumumab has been reported in light-chain amyloidosis achieving rapid and deep responses. The combination of daratumumab, pomalidomide, and dexamethasone (DaraPomD) is particularly interesting for severe AL based on success in multiple myeloma. A 43-year-old female with light-chain amyloidosis and concomitant multiple myeloma presented with severe bowel dysmotility causing abdominal pain, anemia, and a 100-pound unintentional weight loss. A combination of cyclophosphamide, bortezomib, and dexamethasone was initiated but after five cycles her symptoms were progressing and therapy was switched to DaraPomD to optimize response. At the conclusion of two cycles she had achieved an amyloid complete-hematologic response, with her recurring ileus and abdominal pain significantly improved. Additionally, cardiac markers also suggested a rapid response without a common paradoxical worsening of congestive heart failure, and was overall well tolerated. Given the severe symptoms and refractory nature of our patient's disease DaraPomD was reasonable. With the tolerability and response seen, this patient experience supports a formal clinical trial evaluating the safety and efficacy of DaraPomD in light-chain amyloidosis.

scholarly journals Effects of Amyloid Light-chain Amyloidosis on Clinical Characteristics and Prognosis in Multiple Myeloma: A Single-center Retrospective Study

2020 ◽  
Author(s):  
Junhui Xu ◽  
Mangju Wang ◽  
Ye Shen ◽  
Miao Yan ◽  
Weiwei Xie ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Light Chain ◽  
Clinical Characteristics ◽  
Al Amyloidosis ◽  
Term Survival ◽  
Bone Marrow Biopsies ◽  
Poor Prognostic Factor ◽  
Light Chain Amyloidosis ◽  
Amyloid Light Chain

Abstract Background Amyloid light-chain amyloidosis (AL amyloidosis) is commonly associated with multiple myeloma. However, the clinical characteristics and prognosis of multiple myeloma with AL amyloidosis are not particularly clear. Methods Patients with multiple myeloma in the Peking University First Hospital registry from 2010 to 2018 were studied. The clinical and laboratory information were collected from first presentation to death or until the last available clinical follow-up. The patients’ survival and outcomes were analyzed, and the relationships between the clinical parameters and survival were also assessed. Results Compared with patients without AL amyloidosis, patients with AL amyloidosis had higher incidence of BNP ≧ 1000 pg/ml (P = 0.001), ALP > 187.5 IU/L (P = 0.002) and ALB < 35 g/L (P = 0.001), but lower incidence of HB < 85 g/L (P = 0.031), hypercalcemia > 2.65 mmol/L (P = 0.008), bone destruction more than three (P < 0.001), bone marrow plasma cell ratio ≧ 30% (P < 0.001) and worse D-S stage (P < 0.001). Multiple myeloma was more often complicated by λ-type AL amyloidosis than κ-type AL amyloidosis. However, further comparison found that multiple myeloma with κ-type AL amyloidosis had higher incidence of ALP > 187.5 IU/L (P = 0.001) and renal insufficiency (P = 0.001) along with worse D-S stage (P = 0.003) than multiple myeloma with λ-type AL amyloidosis. Renal biopsies of many patients suggested AL amyloidosis, but their bone marrow biopsies or subcutaneous abdominal fat pad aspirates results were negative. The existence of AL amyloidosis especially the heart involvement was related to shorter long-term survival of multiple myeloma according to univariate analysis. Cox regression model for overall survival detected the presence of AL amyloidosis in multiple myeloma having independent prognostic significance (RR = 4.52, P = 0.049). Conclusions Patients with multiple myeloma accompanied by AL amyloidosis have milder target organ damage and lower tumor burden. The incidence of AL amyloidosis in κ-type multiple myeloma is lower but more severe than λ-type multiple myeloma. Renal biopsy can help to identify patients with AL amyloidosis. AL amyloidosis is an independent poor prognostic factor for multiple myeloma is mainly because involvements of important organs especially the heart.

Light chain multiple myeloma with cutaneous AL amyloidosis

2008 ◽  
Vol 6 (9) ◽  
pp. 744-745 ◽  
Author(s):  
Maria Rita Becker ◽  
Rainer Rompel ◽  
Jörg Plum ◽  
Timo Gaiser
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
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 Prevalence and clinical implications of t(11;14) in patients with amyloid light-chain amyloidosis with or without concurrent multiple myeloma

2019 ◽  
Vol 49 (2) ◽  
pp. 195-198 ◽  
Author(s):  
Hiroki Kobayashi ◽  
Yoshiaki Abe ◽  
Daisuke Miura ◽  
Kentaro Narita ◽  
Akihiro Kitadate ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Clinical Implications ◽  
Light Chain Amyloidosis ◽  
Amyloid Light Chain

A pharmacist’s review of the treatment of systemic light chain amyloidosis

2020 ◽  
pp. 107815522096353
Author(s):  
David M. Hughes ◽  
Andrew Staron ◽  
Vaishali Sanchorawala
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Case Reports ◽  
Evidence Base ◽  
Chemotherapeutic Agents ◽  
Plasma Cell Dyscrasia ◽  
High Dose ◽  
Al Amyloidosis ◽  
Tertiary Referral Center ◽  
Treatment Considerations

Objective Systemic light-chain (AL) amyloidosis is an uncommon hematologic plasma cell dyscrasia that is becoming increasingly recognized. Therapeutic agents used in AL amyloidosis overlap with those used in multiple myeloma; however, differences in disease features change treatment efficacy and tolerance. Pharmacists must be cognizant of these distinctions. Herein, this review article provides an up-to-date guide to treatment considerations for systemic AL amyloidosis in both the front-line and relapsed settings. Data sources: A comprehensive literature search was performed using the PubMed/Medline database for articles published through (June 2020) regarding treatments for AL amyloidosis. Search criteria included therapies that are FDA approved for multiple myeloma, as well as investigational agents. This review of chemotherapeutic agents reflects the current clinical practice guidelines endorsed by NCCN along with commentary based on the experience of pharmacists from a tertiary-referral center treating many patients with AL amyloidosis. Data consists of randomized controlled trials, observational cohorts, case reports, and ongoing clinical trials. Data summary: Frontline options discussed here include high-dose melphalan with autologous stem cell transplantation and bortezomib-based regimens. Regarding the relapsed setting, supporting data are compiled and summarized for: bortezomib, ixazomib, carfilzomib, lenalidomide, pomalidomide, daratumumab, elotuzumab, isatuximab, venetoclax, NEOD001, and melflufen. Conclusions The treatment platform for AL amyloidosis is expanding with novel agents traditionally used in multiple myeloma being adopted and modified for use in AL amyloidosis. The pharmacist’s familiarity with the clinical evidence base for these agents and how they fit into standard protocols for AL amyloidosis is critical as dosing and monitoring recommendations are unique from multiple myeloma.

scholarly journals Confocal Cornea Microscopy Detects Involvement of Corneal Nerve Fibers in a Patient with Light-Chain Amyloid Neuropathy Caused by Multiple Myeloma: A Case Report

2016 ◽  
Vol 8 (2) ◽  
pp. 134-139 ◽  
Author(s):  
Dietrich Sturm ◽  
Tobias Schmidt-Wilcke ◽  
Tineke Greiner ◽  
Christoph Maier ◽  
Marc Schargus ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Case Report ◽  
Light Chain ◽  
Nerve Fibers ◽  
Al Amyloidosis ◽  
Fiber Damage ◽  
Small Fiber ◽  
Corneal Nerve ◽  
Al Amyloid

Changes in the subbasal corneal plexus detected by confocal cornea microscopy (CCM) have been described for various types of neuropathy. An involvement of these nerves within light-chain (AL) amyloid neuropathy (a rare cause of polyneuropathy) has never been shown. Here, we report on a case of a patient suffering from neuropathy caused by AL amyloidosis and underlying multiple myeloma. Small-fiber damage was detected by CCM.

scholarly journals Neuropathy and efficacy of once weekly subcutaneous bortezomib in multiple myeloma and light chain (AL) amyloidosis

PLoS ONE ◽  
2017 ◽  
Vol 12 (3) ◽  
pp. e0172996 ◽  
Author(s):  
Surbhi Sidana ◽  
Mayur Narkhede ◽  
Paul Elson ◽  
Debbie Hastings ◽  
Beth Faiman ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Al Amyloidosis

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.

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