How we treat systemic light-chain amyloidosis.

How we treat systemic light-chain amyloidosis. Clin Adv Hematol Oncol. 2015 May;13(5):315-24 Authors: Chaulagain CP, Comenzo RL Abstract Systemic light-chain (AL) amyloidosis is a multisystem disease characterized by organ toxicity and damage due to monoclonal free light chains, which are produced by a neoplastic clone of plasma cells in bone marrow. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains and thereby stop or reverse organ toxicity and damage. AL amyloidosis remains a formidable and often incurable disease despite treatment options that include corticosteroids, cytotoxic chemotherapy, risk-adapted melphalan, autologous hematopoietic stem cell transplantation, proteasome inhibitors, and immunomodulatory drugs. New and effective treatment approaches that can reverse the organ damage are urgently needed. Physicians and clinical staff should be aware of the importance of providing best supportive care to patients with advanced AL-related organ dysfunction, given the patients' often tenuous hemodynamics and fragile functional status. Organ transplantation has a role in selected clinical situations, and the treating hematologist should be aware of this sometimes-useful option. PMID: 26352777 [PubMed - in process]
Source: Clinical Advances in Hematology and Oncology - Category: Cancer & Oncology Tags: Clin Adv Hematol Oncol Source Type: research

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Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
AbstractFamilial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are ...
Source: Rheumatology International - Category: Rheumatology Source Type: research
CONCLUSION: NT-proBNP may be a sensitive but nonspecific biomarker for assessing CA. However, CMR is the only imaging modality that can assess the pathophysiologic background of cardiac hypertrophy and the severity of CA, irrespective of NT-proBNP level. PMID: 31796976 [PubMed - as supplied by publisher]
Source: Herz - Category: Cardiology Tags: Herz Source Type: research
Authors: Crawford DC, Lin J, Cooke Bailey JN, Kinzy T, Sedor JR, O'Toole JF, Bush WS Abstract Return of results is not common in research settings as standards are not yet in place for what to return, how to return, and to whom. As a pioneer of large-scale of return of research results, the Precision Medicine Initiative Cohort now known of All of Us plans to return pharmacogenomic results and variants of clinical significance to its participants starting late 2019. To better understand the local landscape of possibilities regarding return of research results, we assessed the frequency of pathogenic variants and APO...
Source: Pacific Symposium on Biocomputing - Category: Bioinformatics Tags: Pac Symp Biocomput Source Type: research
ont C Abstract Surgery of the cervical spine under conscious sedation has been rarely reported in the literature. The main indications are the lack of neurophysiological monitoring and surgery in patients with high cardiovascular risk. To date, no reports of awake C1-2 instrumentation have been published in the English-language literature. The authors present the case of a 76-year-old patient with multiple myeloma and severe cardiomyopathy associated with primary amyloidosis who experienced severe myelopathy from a C2 pseudotumor associated with an odontoid fracture. Due to his high cardiovascular risk, the patien...
Source: Journal of Neurosurgery.Spine - Category: Neurosurgery Authors: Tags: J Neurosurg Spine Source Type: research
Publication date: Available online 9 November 2019Source: Journal of Molecular BiologyAuthor(s): Tyler M. Marcinko, Chungwen Liang, Sergey Savinov, Jianhen Chen, Richard W. VachetAbstractIn dialysis patients, the protein β2-microglobulin (β2m) forms amyloid fibrils in a condition known as dialysis-related amyloidosis. To understand the early stages of the amyloid assembly process, we have used native electrospray ionization (ESI) together with ion mobility mass spectrometry (IM-MS) to study soluble preamyloid oligomers. ESI-IM-MS reveals the presence of multiple conformers for the dimer, tetramer, and hexamer tha...
Source: Journal of Molecular Biology - Category: Molecular Biology Source Type: research
Researchers here argue that fibrosis of cardiac tissue is an important contribution to the development of atrial fibrillation in older patients. Fibrosis is a feature of many age-related conditions, a dysfunction in tissue maintenance processes that involves the generation of scar-like deposits of collagen by overactive fibroblasts. This scarring disrupts normal tissue structure and function in many organs, including the heart, and there is no good approved therapy to treat the progression of fibrosis: even slowing it is haphazard and unreliable. This may soon change. Fibrosis appears to be caused to a large degree ...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs
Leukemia, Published online: 03 December 2019; doi:10.1038/s41375-019-0640-4Light chain amyloidosis induced inflammatory changes in cardiomyocytes and adipose-derived mesenchymal stromal cells
Source: Leukemia - Category: Hematology Authors: Source Type: research
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an underappreciated cause of heart failure that results from misfolded TTR (prealbumin) protein. Diflunisal is an approved non-steroidal anti-inflammatory drug (NSAID) that stabilizes TTR, with limited data available regarding effects on cardiac structure and function.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Source Type: research
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