Publications by authors named "V Sanchorawala"
Background: Each monoclonal antibody light chain associated with AL amyloidosis has a unique sequence. Defining how these sequences drive amyloid deposition could facilitate faster diagnosis and lead to new treatments.
Methods: Light chain sequences are collected in the AL-Base repository.
Article Synopsis
- - A study compared the outcomes of autologous hematopoietic stem cell transplantation (AHSCT) using unmodified peripheral blood stem cells (PBSC) with maintenance therapy (mycophenolate mofetil) versus a previously studied method that involved CD34+ selected PBSC in patients with diffuse systemic sclerosis (SSc).
- - In this trial involving 20 high-risk SSc patients, the 5-year overall survival (OS) was estimated at 85%, and event-free survival (EFS) was 75%, indicating promising results with this new approach.
- - The research highlighted a potential risk factor for poor outcomes in patients undergoing AHSCT: having a low estimated glomerular filtration rate (
Antibody light chains form amyloid fibrils that lead to progressive tissue damage in amyloid light chain (AL) amyloidosis. The properties of each patient's unique light chain appear to determine its propensity to form amyloid. One factor is N-glycosylation, which is more frequent in amyloid-associated light chains than in light chains from the normal immune repertoire.
View Article and Find Full Text PDFBackground: Circulating cardiac troponin-I (cTnI) plays a crucial role in biomarker staging systems, offering important information for prognostification and risk stratification of patients with AL amyloidosis. High-sensitivity cTnI (HS-cTnI) assays have been introduced in practice; however, the data on the concordance between conventional and HS-cTnI and the utility of HS-cTnI in cardiac biomarker staging are lacking.
Methods: Seventy-eight consecutive patients with AL amyloidosis who were prospectively evaluated at the Boston University Amyloidosis Center from October 2022 through March 2023 were included.
Light-chain (AL) amyloidosis for nephrologists-treatment standard.
Nephrol Dial Transplant
December 2024
Article Synopsis
- Amyloidosis is a complex group of diseases caused by proteins misfolding into amyloid fibrils, with AL amyloidosis being the most common type related to plasma cell light chains.
- Increased awareness of AL amyloidosis has led to higher reported cases, particularly affecting the kidneys in about 70% of patients, which significantly impacts their quality of life.
- Effective management of AL amyloidosis requires early detection, proper staging, and an understanding of the latest treatment options, along with their potential side effects, especially for kidney health.