Improving management of AL amyloidosisdoi:10.47102/annals-acadmedsg.2023370Yuh Shan LeeHuang, JeffreyAnnals of the Academy of Medicine, Singapore
Summary Immunoglobulin light chain (AL) amyloidosis, the most common of the systemic amyloidosis, is characterized by the deposition of amyloid fibrils that derive from the aggregation of misfolded monoclonal immunoglobulin light chains. Amyloid fibrils disrupt tissue architecture and the pre-fibril oligome...
Cardiac amyloidosis is a potentially deadly disease characterized by progressive infiltration of amyloid fibrils, and it is increasingly recognized as an underdiagnosed but important cause of heart failure. Given its unique pathogenesis, there are key differences in the management of cardiac amyloidosis ...
Amyloidosis is characterized by extracellular deposition of abnormal protein, consisting of primary, secondary, hemodialysis-related, hereditary, senile and localized type. Primary amyloidosis is associated with monoclonal light chains. Secondary amyloidosis is associated with inflammatory, infectious, and ...
Transthyretin (TTR) amyloidosis (ATTR amyloidosis) is a multisystemic, multigenotypic disease resulting from deposition of insoluble ATTR amyloid fibrils in various organs and tissues. Although considered rare, the prevalence of this serious disease is likely underestimated because symptoms can be non-spe...
Additionally, paraneoplastic thrombophilia can result in severe neurological deficits due to ischemic strokes, and tumor-associated amyloidosis can lead to the formation of pathological protein aggregates in and around nerve structures. Another example of indirect complications of malignant diseases are ...
Rajkumar SV, Mesa RA, Fonseca R, Schroeder G, Plevak MF, Dispenzieri A, et al. Bone marrow angiogenesis in 400 patients with monoclonal gammopathy of undetermined significance, multiple myeloma, and primary amyloidosis. Clin Cancer Res. 2002;8(7):2210–6. PubMed Google Scholar Rajkumar SV...
Heart failure with preserved ejection fraction (HFpEF) has grown to become the dominant form of heart failure worldwide, in tandem with ageing of the general population and the increasing prevalences of obesity, diabetes mellitus and hypertension. The cl
Differential diagnosis of adult onset HCM should include a complete work-up, in order to exclude potentially treatable HCM-mimics as cardiac amyloidosis (CA) and Fabry disease (FD) [19,66] (Fig. 3). Although its prevalence is uncertain, CA is overlooked in many clinical scenarios. Recent aut...
The cause of sudden death may be cardiac arrhythmia associated with structural changes in the heart – rheumatoid nodules, amyloidosis, and ischemic heart disease [4]. Electrocardiography should be performed and, in the case of symptoms of heart disease or risk factors, echocardiography may be ...