A5044434947- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Neuroendocrine Tumor Research Advances
- Dermatological and Skeletal Disorders
- Sarcoidosis and Beryllium Toxicity Research
- Alzheimer's disease research and treatments
- IgG4-Related and Inflammatory Diseases
- Eosinophilic Disorders and Syndromes
- Pancreatitis Pathology and Treatment
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Pituitary Gland Disorders and Treatments
- Cardiovascular Syncope and Autonomic Disorders
- Trace Elements in Health
- Chronic Lymphocytic Leukemia Research
- Medical Imaging and Pathology Studies
- Drug Transport and Resistance Mechanisms
- Protein Interaction Studies and Fluorescence Analysis
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Pregnancy and Medication Impact
- Pneumocystis jirovecii pneumonia detection and treatment
- Chronic Myeloid Leukemia Treatments
Boston University
2020-2024
Boston Medical Center
2020-2024
Amyloidosis Foundation
2021-2024
Abstract The recent decades have ushered in considerable advancements the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends primary causes death among 2337 individuals with amyloidosis referred to Boston University Amyloidosis Center. Outcomes were analyzed according date diagnosis: 1980-1989 (era 1), 1990-1999 2), 2000-2009 3), 2010-2019 4). Overall...
High-dose melphalan and stem cell transplantation (HDM/SCT) is an effective treatment for selected patients with AL amyloidosis. We report the long-term outcomes of 648 amyloidosis treated HDM/SCT over 25 years. Hematologic CR was achieved by 39% patients. The median duration hematologic 12.3 years, 45% a had no evidence recurrent plasma dyscrasia at 15 years after HDM/SCT. With follow-up interval 8 event-free survival (EFS) overall (OS) were 3.3 7.6 respectively. Patients OS 30% these...
Patients with advanced cardiac immunoglobulin light chain (AL) amyloidosis have a poor prognosis. Early hematologic and responses can prolong survival, but predictors of these outcomes yet to be clarified. We report on 142 patients newly diagnosed stage IIIb AL amyloidosis. After median follow-up 60 months, the overall survival (OS) was 9 months. Independent baseline factors associated shorter OS were symptom onset diagnosis >6 months (hazard ratio [HR], 1.94; P = .003); bone marrow...
Abstract Introduction Symptomology of AL amyloidosis can be vague, with a broad range manifestations and potential etiologies. We sought to determine whether time from initial patient‐reported symptom onset diagnosis was associated survival. Methods The Boston University Amyloidosis Patient Database queried for patients who presented the Center evaluation 2010 2015. Results A total 324 were evaluated evaluation. median 7.1 months (range, 0‐61). At data cutoff, 60.2% (n = 195) alive; those,...
Summary Patients with AL amyloidosis can have persistent organ dysfunction despite achieving a haematological complete response (hemCR). We aimed to identify factors for non‐response among 143 patients who achieved hemCR ≥6 months. Kidney, heart and liver were observed in 40/117 (34%), 19/68 (28%) 3/17 (18%) respectively. Predisposing varied by system. Kidney non‐responders had more advanced at diagnosis, whereas disproportionately lambda‐typic amyloidogenic light chains. Most without an...
Diagnostic algorithms for amyloidosis have evolved over the past decade, particularly with incorporation of imaging-based techniques to detect amyloid cardiomyopathy. We sought identify key sources misidentification in community, which lead false positive referrals a tertiary centre.We conducted retrospective review all Amyloidosis Centre from 2010 2021 and identified cases lacking pathology upon final adjudication after extensive assessment at centre. Factors were examined.Among 2409...
Abstract Immunoglobulin light chain ( AL ) amyloidosis is a multisystem disease with varied treatment options and disease‐related outcomes. Current staging systems rely on limited number of cardiac, renal, plasma cell dyscrasia biomarkers. To improve prognostication for all‐cause mortality end‐stage kidney ESKD ), we applied unsupervised machine learning using comprehensive set clinical laboratory parameters. Our study cohort comprised 2067 patients newly diagnosed, biopsy‐proven from the...
Test the feasibility of replacing 24-hour urine collection with a single voided urinary protein-creatinine ratio (UPCR) in patients amyloid light-chain (AL) amyloidosis.Retrospective study examining correlation between measurement and UPCR at various proteinuria levels using linear regression analysis Pearson's coefficient (r). We assessed how these 2 different measurements would alter diagnosis, staging, kidney response assessment AL amyloidosis.We included 265 systemic amyloidosis who...
Background β2-microglobulin amyloidosis was first described in the 1980s as a protein deposition disease associated with long-term haemodialysis. More recently, two inherited forms resulting from separate point mutations gene have been identified. In this report, we detail novel β2M variant, P32L, caused by unique dinucleotide mutation that is linked to systemic hereditary amyloidosis.Methods Three family members Portuguese kinship featured cardiomyopathy, requiring organ transplantation one...
Abstract Background 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 practice; however, the data on concordance between conventional HS-cTnI utility are lacking. Methods Seventy-eight consecutive amyloidosis who were prospectively evaluated at Boston University Amyloidosis Center from...