Pilot Study of Bortezomib and Dexamethasone Pre- and Post-Risk-Adapted Autologous Stem Cell Transplantation in AL Amyloidosis

Immunoglobulin light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by production and deposition of misfolded immunoglobulin light chains into target tissues, leading to end-organ damage. Most therapeutic interventions are adapted from experience in multiple myeloma (MM) and directed at eradicating the pathologic plasma cells that produce circulating amyloidogenic free light chains. However, unlike in MM, in AL amyloidosis therapeutic choice is limited by the enhanced susceptibility to toxicity of organs affected by amyloid deposition.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Tags: Brief Article Source Type: research

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Immunoglobulin light chain amyloidosis (AL) is a plasma cell (PC) dyscrasia characterized by a neoplastic malignant PC population producing highly toxic light chains. Despite overlapping features with multiple myeloma (MM), therapeutic options are limited as a result of disease-specific treatment toxicities. Given delays in diagnosis, patients often present with advanced disease, making them poor candidates for autologous stem-cell transplantation.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Case Report Source Type: research
Publication date: Available online 1 April 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Divaya Bhutani, Siyang Leng, Suzanne LentzschAbstractImmunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder leading to the production and extracellular deposition of abnormal immunoglobulin light chains called amyloid. The pathogenesis of the disorder is driven by an abnormal plasma cell clone producing excessive monoclonal immunoglobulin light chains, which undergo deposition in various organs of the body such as the heart, kidney, and gastrointestinal tract. The outcome of the disease remains...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Acute kidney injury (AKI) is a common complication after allogeneic stem cell transplantation (ASCT); however, its incidence and outcome in patients transplanted for multiple myeloma (MM) is unknown. Our results suggest that AKI occurs with low incidence and reduced severity after ASCT for MM. Prophylactic measures in patients with preexisting –kidney failure may further reduce this risk. AbstractAcute kidney injury (AKI) is a common complication after allogeneic stem cell transplantation; however, its incidence and outcome in patients transplanted for multiple myeloma (MM) is unknown. We evaluated the incidence, sev...
Source: Cancer Medicine - Category: Cancer & Oncology Authors: Tags: ORIGINAL RESEARCH Source Type: research
Publication date: Available online 1 April 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Divaya Bhutani, Siyang Leng, Suzanne LentzschAbstractAL amyloidosis is a plasma cell disorder leading to the production and extracellular deposition of abnormal immunoglobulin light chains called amyloid. The pathogenesis of the disorder is driven by an abnormal plasma cell clone producing excessive monoclonal immunoglobulin light chains which undergo deposition in various organs of the body such as heart, kidney and gastrointestinal tract. The outcome of the disease remains poor with significant morbidity and mortality ...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
This article covers the mechanism of action, pharmacology and clinical trial results of ixazomib while under investigation for the treatment of various lymphoproliferative disorders. We examine the findings from several phase 3 clinical trials (i) the pivotal TOURMALINE-MM1 study investigating ixazomib versus placebo in combination with lenalidomide and dexamethasone (ii) the TOURMALINE-MM3 study investigating ixazomib versus placebo as a maintenance therapy in newly-diagnosed MM following induction therapy and autologous stem cell transplantation,(iii) the TOURMALINE-MM2 study investigating ixazomib versus placebo in comb...
Source: Expert Opinion on Investigational Drugs - Category: Drugs & Pharmacology Tags: Expert Opin Investig Drugs Source Type: research
We report a rare case of AL amyloidosis coincidence with psoriasis obtained continuous complete remission of the 2 diseases by autologous hematopoietic stem cell transplantation (ASCT). Patient concerns: A 58-year-old man with a 30-year history of psoriasis complaining of edema and hypotension for 2 weeks was referred to our institution. His urine protein was quantified 2.83 g/day, without hematuria and decrease of glomerular filtration rate. Diagnosis: Renal biopsy confirmed AL amyloidosis and multiple myeloma was excluded by bone marrow cytomorphologic examination. Interventions: Chemotherapy regimen based on ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
In conclusion, patients with normalization of different parameters related to FLC at 2 months of the onset of treatment exhibit a deeper and prolonged response to bortezomib-based chemotherapies, suggesting that this assessment could be of paramount interest in the early prediction of outcomes in patients with MM receiving treatment. Therefore, early FLC determinations should be incorporated into prospective clinical trials to validate these observations.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 651. Myeloma: Biology and Pathophysiology, excluding Therapy: Poster I Source Type: research
CONCLUSIONS: The SAVE trial may enable early appreciation of risk of AL -type based on the variable region germline gene used by the clonal plasma cells. In MGUS and SMM patients with elevated lambda FLC, below normal ratios, and dFLC> 23mg/L, the clonal gene can be identified by RT-PCR with CD138-selected cells from a single peripheral blood specimen 70% of the time. Close follow up of patients at risk may reduce the likelihood of eventually being diagnosed with advanced organ involvement. Earlier diagnosis may permit prompt induction therapy if required and expanded application of MEL 200 SCT, a preferred treatment for patients with
Source: Blood - Category: Hematology Authors: Tags: 651. Myeloma: Biology and Pathophysiology, excluding Therapy: Poster I Source Type: research
Introduction: Prognosis of AL amyloidosis has improved in recent years; however for many patients prognosis remains poor. We aimed to define patient-, disease- and treatment characteristics which are associated with long-term survival.Method: A retrospective chart review of all patients with biopsy-proven systemic AL amyloidosis, who were seen within 90 days of the confirmed diagnosis. Long-term survival was defined as 5-year and 10-year survival from the time of diagnosis. For 5-year survival we selected patients seen between January 1, 2000 and December 31, 2012 (allowing a minimum of 5-year follow-up, n=1331) and for 10...
Source: Blood - Category: Hematology Authors: Tags: 653. Myeloma: Therapy, excluding Transplantation: Poster II Source Type: research
Conclusions]The ORR after 4 cycles ofmodifiedRVD-lite was high (91.1%). These results might be related to low rate of discontinuation of treatment. Also, the ORR after ASCT was comparable to the results previously published (N Engl J Med .2017 Apr 6:376(14):1311). AEs in modified RVD-lite were feasible and manageable in most patients. Our results suggest that modifiedRVD-lite is very feasible and effective treatment for patients with transplant eligible NDMM.DisclosuresSuzuki: Sanofi Aventis: Consultancy, Honoraria; Takeda: Consultancy, Honoraria; Ono: Consultancy, Honoraria; Bristol-Myers Squibb: Consultancy, Honoraria; N...
Source: Blood - Category: Hematology Authors: Tags: 653. Myeloma: Therapy, excluding Transplantation: Poster I Source Type: research
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