Download full-text PDF

Source
http://dx.doi.org/10.1159/000494425DOI Listing

Publication Analysis

Top Keywords

bortezomib rescue
4
rescue comment
4
comment article
4
article nachmias
4
nachmias entitled
4
entitled bortezomib-based
4
bortezomib-based protocol
4
protocol induces
4
induces high
4
high rate
4

Similar Publications

Epstein-Barr virus (EBV) manipulates the ubiquitin-proteasome system and regulators of Bcl-2 family to enable the persistence of the virus and survival of the host cells through the expression of viral proteins in distinct latency patterns. We postulate that the combination of bortezomib (proteasome inhibitor) and venetoclax (Bcl-2 inhibitor) [bort/venetoclax] will cause synergistic killing of post-transplant lymphoproliferative disorder (PTLD) through targeting the pro-survival function of latent viral proteins such as latent membrane protein-1 (LMP-1) and EBV nuclear antigen-3C (EBNA-3C). Bort/venetoclax could synergistically kill spontaneous lymphoblastoid cell lines (sLCLs) derived from patients with PTLD and EBV-associated hemophagocytic lymphohistiocytosis by inducing DNA damage response, apoptosis and G1-S cell cycle arrest in a ROS-dependent manner.

View Article and Find Full Text PDF

Purpose Of Review: This review aims to assess the therapeutic strategies available for relapsed/refractory patients with immunoglobulin light chain (AL) amyloidosis who received upfront daratumumab-based regimens.

Recent Findings: The treatment landscape of AL amyloidosis has changed radically thanks to the introduction in the upfront setting of daratumumab in combination with bortezomib, cyclophosphamide and dexamethasone (DaraCyBorD) which improved patients' outcomes increasing the rate of hematologic and organ responses. However, many patients eventually relapse or are refractory to daratumumab and the best salvage therapy is not well defined yet.

View Article and Find Full Text PDF

Response matters in light chain amyloidosis, whatever it takes.

Br J Haematol

July 2024

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Access to upfront daratumumab for AL amyloidosis is expanding, but it is not universal. Bomsztyk et al. show that patients who do not receive front-line daratumumab can be effectively rescued with this agent, indicating that deep haematological response should be pursued tenaciously.

View Article and Find Full Text PDF

Introduction: High sustained anti-rhGAA antibody titers (HSAT; ≥12,800) are directly linked to reduced efficacy of enzyme replacement therapy (ERT) and subsequent clinical deterioration in infantile-onset Pompe disease (IOPD). We have previously demonstrated the safety and effectiveness of a bortezomib-based immune-tolerance induction (ITI) regimen (bortezomib, rituximab, methotrexate, and IVIG) in eliminating HSAT.

Methods: Here, we describe two IOPD cases (patients 6 and 8) who developed HSAT at 8 and 10 weeks on ERT despite transient low-dose methotrexate ITI administration in the ERT-naïve setting and were treated with a bortezomib-based ITI regimen, and we compare their courses to a series of six historical patients (patients 1-5, and 7) with a similar presentation who exemplify our evolving approach to treatment.

View Article and Find Full Text PDF
Article Synopsis
  • * The results showed significant improvement in patient responses, with complete or better responses increasing from 19.9% after induction to 62.4% after maintenance, alongside a 3-year progression-free survival rate of 83.5% and overall survival rate of 92.5%.
  • * While the treatment was tolerable, around 30% of patients experienced severe side effects, and those with high-risk cytogenetics
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!