Impact of Hematopoietic Cell Transplantation Comorbidity Index (HCT-CI) on AL Amyloidosis Outcomes
Autologous stem cell transplantation (ASCT) remains the preferred standard consolidation therapy for patients with AL amyloidosis. Whilst several factors are known to impact outcomes, conventional risk scores for transplant-related mortality such as the HCT-CI have not been fully explored in this setting. (Source: Biology of Blood and Marrow Transplantation)
Source: Biology of Blood and Marrow Transplantation - February 1, 2024 Category: Hematology Authors: Portia Smallbone, Supawee Saengboon, Qaiser Bashir, Blessie Elizabeth Nelson, Jeremy Ramdial, Neeraj Y. Saini, Chitra Hosing, Uday R. Popat, Ruby Delgado, Melody Becnel, Hans C. Lee, Robert Orlowski, Elizabeth J. Shpall, Richard E. Champlin, Muzaffar H. Q Tags: 533 Source Type: research

Atrial electrofunctional predictors of incident atrial fibrillation in cardiac amyloidosis
Atrial fibrillation (AF) is common in patients with cardiac amyloidosis (CA) and is a significant risk factor for heart failure hospitalization and thromboembolic events. (Source: Heart Rhythm)
Source: Heart Rhythm - February 1, 2024 Category: Cardiology Authors: Giulio Sinigiani, Laura De Michieli, Aldostefano Porcari, Chiara Zocchi, Anna Sorella, Carlotta Mazzoni, Giandomenico Bisaccia, Antonio De Luca, Gianluca Di Bella, Dario Gregori, Federico Perfetto, Marco Merlo, Gianfranco Sinagra, Sabino Iliceto, Martina Source Type: research

Healthcare resource utilization and cost-of-illness in systemic light chain (AL) amyloidosis in Europe: results from the real-world, retrospective EMN23 study
Systemic light chain (AL) amyloidosis is a rare clonal disease caused by immunoglobulin light chains that misfold, aggregate, and form amyloid fibrils in several critical organ systems, predominantly in the heart and kidneys ( ∼70% of patients). 1,2 The crude incidence of AL amyloidosis was 10.44 patients per million population in 2018. 3 (Source: Clinical Lymphoma, Myeloma and Leukemia)
Source: Clinical Lymphoma, Myeloma and Leukemia - January 31, 2024 Category: Hematology Authors: Arnaud Jaccard, Frank Bridoux, Wilfried Roeloffzen, Monique C. Minnema, Rui Bergantim, Roman H ájek, Cristina João, M. Teresa Cibeira, Giovanni Palladini, Stefan Schönland, Giampaolo Merlini, Paolo Milani, Meletios A. Dimopoulos, Sriram Ravichandran, U Source Type: research

Too young for an acquired cardiomyopathy? Cobalt metallosis as a cardiac amyloidosis mimicker
We report the case of a young female referred to our centre for the suspicion of cardiac amyloidosis presenting with low electrocardiogram voltage, left ventricular hypertrophy, pericardial effusion, and global and longitudinal systolic impairment with apical sparing pattern. Her medical history was remarkable for arthroplasty in the context of congenital hip dysplasia. Two years prior to presentation, she underwent revision surgery for prosthesis malfunction, and tissue metallosis was initially documented. At the current presentation, cobalt metallosis was confirmed, as the circulating cobalt and chromium levels were seve...
Source: ESC Heart Failure - January 30, 2024 Category: Cardiology Authors: Mihnea Casian, Ramona Bica, Virgil Ionescu, Vlad Predescu, Radu Țincu, Ruxandra Jurcuț Tags: Case Report Source Type: research

Hereditary transthyretin amyloidosis presenting with carpal tunnel syndrome
CMAJ. 2024 Jan 28;196(3):E95-E99. doi: 10.1503/cmaj.230671.NO ABSTRACTPMID:38286493 | PMC:PMC10833096 | DOI:10.1503/cmaj.230671 (Source: cmaj)
Source: cmaj - January 29, 2024 Category: General Medicine Authors: Nicole Zaki Nicholas J Miller Patrick Frosk Aditya Sharma Source Type: research

Hereditary transthyretin amyloidosis presenting with carpal tunnel syndrome
CMAJ. 2024 Jan 28;196(3):E95-E99. doi: 10.1503/cmaj.230671.NO ABSTRACTPMID:38286493 | PMC:PMC10833096 | DOI:10.1503/cmaj.230671 (Source: Canadian Medical Association Journal)
Source: Canadian Medical Association Journal - January 29, 2024 Category: General Medicine Authors: Nicole Zaki Nicholas J Miller Patrick Frosk Aditya Sharma Source Type: research

Aetiology, ejection fraction and mortality in chronic heart failure: a mediation analysis
Conclusions The direct effect of aetiology on mortality dominates the indirect effect through LVEF. Therefore, clarification of aetiology is as important as measurement of LVEF. (Source: Heart)
Source: Heart - January 29, 2024 Category: Cardiology Authors: Fritz, J., Belovari, K., Ulmer, H., Zaruba, M.-M., Messner, M., Ungericht, M., Siebert, U., Ruschitzka, F., Bauer, A., Poelzl, G. Tags: Heart failure and cardiomyopathies Source Type: research

Deep phenotyping of p.(V142I) ‐associated variant transthyretin amyloid cardiomyopathy: Distinct from wild‐type transthyretin amyloidosis?
Conclusionp.(V142I)-ATTRv-CM has an aggressive phenotype characterized by myocyte loss and widespread myocardial infiltration which may account for frequent biventricular failure and poor prognosis in this ATTR-CM genotypic subgroup. (Source: European Journal of Heart Failure)
Source: European Journal of Heart Failure - January 28, 2024 Category: Cardiology Authors: Yousuf Razvi, Adam Ioannou, Rishi K. Patel, Liza Chacko, Nina Karia, Mattia Riefolo, Aldostefano Porcari, Muhammad Umaid Rauf, Neasa Starr, Sashiananthan Ganesananthan, Iona Blakeney, Nandita Kaza, Stefano Filisetti, Roos Eline Bolhuis, Doro Tags: Research Article Source Type: research

A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease
Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocula... (Source: BMC Ophthalmology)
Source: BMC Ophthalmology - January 26, 2024 Category: Opthalmology Authors: Natsuyo Yoshida-Hata, Masashi Mimura, Miwa Aikawa and Tomoyuki Kashima Tags: Case Report Source Type: research

Assessment of Tp-Te interval in patients with cardiac AL amyloidosis
CONCLUSION: We present the first strong evidence of prolonged Tp-Te intervals in patients with cardiac AL amyloidosis. There may also be a relationship between prolonged Tp-Te interval and the development of arrhythmia in this patient group, as in some other groups. There is a need for prospective studies examining the relationship of prolonged Tp-Te interval with arrhythmias and its prognostic significance in cardiac AL amyloidosis.PMID:38270525 | DOI:10.5830/CVJA-2023-059 (Source: Cardiovascular Journal of Africa)
Source: Cardiovascular Journal of Africa - January 25, 2024 Category: Cardiology Authors: Yavuz B Tor Ismat Habibov Mustafa Altinkaynak Mehmet Aydogan Derya Baykiz Mehmet Tayfur Mehmet G Gonenli Imran Onur Sevgi Kalayoglu-Besisik Bulent Saka Sebile N Erten Timur S Akpinar Source Type: research

Tolerability and effectiveness of beta-blockers in patients with cardiac amyloidosis: A systematic review and meta-analysis
This systematic review aimed to assess the tolerability of patients with cardiac amyloidosis (CA) to beta-blockers (BBs) and evaluate its association with adverse outcomes. (Source: International Journal of Cardiology)
Source: International Journal of Cardiology - January 24, 2024 Category: Cardiology Authors: Yu Kang, Nan Qu, Zhongyin Zhang, Qing Zhang, Xiaojing Chen, M.D. Michael Fu Tags: Review Article Source Type: research

Race, Genotype, and Prognosis in Black Patients with Transthyretin Cardiac Amyloidosis
Previous studies suggest worse outcomes in patients with variant transthyretin cardiac amyloidosis due to V122I (ATTRv-CA) compared to patients with wild-type disease (ATTRwt-CA). Given V122I is almost exclusively found in Black patients, it is unclear if this is attributable to the biology of genotype or racial differences. Patients with transthyretin cardiac amyloidosis (ATTR-CA) diagnosed between January 2001 and August 2021 were characterized into 3 categories: (1) White with ATTRwt-CA (White- WT); (2) Black with V122I ATTRv-CA (Black-V122I), and (3) Black with ATTRwt-CA (Black-WT). (Source: The American Journal of Cardiology)
Source: The American Journal of Cardiology - January 24, 2024 Category: Cardiology Authors: Rola Khedraki, Joshua Saef, Pieter Martens, Trejeeve Martyn, Lidiya Sul, Rory Hachamovitch, Lauren Ives, Jerry D. Estep, W. H. Wilson Tang, Mazen Hanna Source Type: research

Relapsed systemic light chain amyloidosis – in search of a higher bar
Bone Marrow Transplantation, Published online: 23 January 2024; doi:10.1038/s41409-024-02203-8Relapsed systemic light chain amyloidosis – in search of a higher bar (Source: Bone Marrow Transplantation)
Source: Bone Marrow Transplantation - January 23, 2024 Category: Hematology Authors: Muhamed Baljevic Salyka Sengsayadeth Source Type: research

Silver-positive kidney AL amyloidosis
J Nephrol. 2024 Jan 22. doi: 10.1007/s40620-023-01827-1. Online ahead of print.NO ABSTRACTPMID:38252262 | DOI:10.1007/s40620-023-01827-1 (Source: Journal of Nephrology)
Source: Journal of Nephrology - January 22, 2024 Category: Urology & Nephrology Authors: Francesco Fontana Gaetano Alfano Laura Verga Riccardo Magistroni Gabriele Donati Source Type: research

Myocardial perfusion in cardiac amyloidosis
ConclusionCardiac amyloidosis is associated with severe inducible myocardial ischaemia demonstrable by histology and CMR stress perfusion mapping. Histological evaluation indicates a complex pathophysiology, where in addition to systolic and diastolic dysfunction, amyloid infiltration of the epicardial arteries and disruption and rarefaction of the capillaries play a role in contributing to myocardial ischaemia. (Source: European Journal of Heart Failure)
Source: European Journal of Heart Failure - January 22, 2024 Category: Cardiology Authors: Liza Chacko, Tushar Kotecha, Adam Ioannou, Niket Patel, Ana Martinez ‐Naharro, Yousuf Razvi, Rishi Patel, Paolo Massa, Lucia Venneri, James Brown, Aldostefano Porcari, Kristopher Knott, Charlotte Manisty, Daniel Knight, Tim Lockie, Roby R Tags: Research Article Source Type: research