A5020463060- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Dermatological and Skeletal Disorders
- Neuroendocrine Tumor Research Advances
- Sarcoidosis and Beryllium Toxicity Research
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- IgG4-Related and Inflammatory Diseases
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Pancreatitis Pathology and Treatment
- Eosinophilic Disorders and Syndromes
- Alzheimer's disease research and treatments
- Pharmacovigilance and Adverse Drug Reactions
- Ocular Diseases and Behçet’s Syndrome
- Acute Ischemic Stroke Management
- Chronic Lymphocytic Leukemia Research
- Health Systems, Economic Evaluations, Quality of Life
- Surgical Simulation and Training
- Cardiovascular Syncope and Autonomic Disorders
- Multiple Myeloma Research and Treatments
- Retinal and Optic Conditions
- Pituitary Gland Disorders and Treatments
- Trace Elements in Health
- Pneumocystis jirovecii pneumonia detection and treatment
- Cerebral Venous Sinus Thrombosis
- Cerebrospinal fluid and hydrocephalus
Boston Medical Center
2018-2025
Boston University
2018-2025
Amyloidosis Foundation
2018-2024
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...
Background Accurate tissue typing in amyloidosis is essential to provide appropriate therapy for individual patients.
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...
Objective: To characterize the changing spectrum of amyloidosis classes, as well patient demographics, at a major US referral centre. Patients and methods: A retrospective analysis was conducted all referrals to Amyloidosis Centre Boston University Medical over last 3 decades. Results: total 3987 new patients with were evaluated between 1990 2018 average number cases per year increasing 2.5-fold during this period. Systemic immunoglobulin light-chain (AL) decreased in proportion each decade...
High-dose melphalan and autologous stem cell transplantation (HDM/SCT) have been used in patients with immunoglobulin light chain (AL) amyloidosis for over 2 decades now durable responses, prolonged survival, decreasing treatment-related mortality. Historically, poorer baseline functional status, advanced age, renal compromise, cardiac involvement treated a risk-adapted modified conditioning dose of (mHDM) 100 to 140 mg/m2 before SCT. In part because these characteristics, receiving mHDM/SCT...
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...
Serum free light chains (sFLC) are independent prognostic markers of disease in chain (AL) amyloidosis, and used the haematologic response criteria for treatment. However, up to 20% patients have low sFLCs at diagnosis, with a difference between involved uninvolved (dFLC) less than 50 mg/L, making responses treatment difficult evaluate. In order characterize this distinct subgroup patients, we retrospectively analyzed 123 AL amyloidosis dFLC <50 mg/L who were diagnosed 2002 2013. 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...
Hereditary gelsolin (AGel) amyloidosis is a systemic disease that characterised by neurologic, ophthalmologic, dermatologic, and other organ involvements. We describe the clinical features with focus on neurological manifestations in cohort of patients AGel referred to Amyloidosis Centre United States.Fifteen were included study between 2005 2022 permission Institutional Review Board. Data collected from prospectively maintained database, electronic medical records telephone...