Articles: amyloidosis.
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Light chain (AL) amyloidosis is a potentially fatal disease of monoclonal plasma cells that leads to accumulation of light chain amyloid fibrils, organ damage, and the manifestations of clinical disease. Meanwhile, coronavirus disease 2019 (COVID-19) is a disease caused by infection with the severe acute respiratory syndrome coronavirus 2 virus, with the potential to cause severe systemic illness and death. ⋯ This overlap creates unique challenges in caring for patients with AL amyloidosis, which are further compounded by the immunosuppressive nature of anti-plasma cell therapies, the need for frequent clinical assessments, and the exclusion of AL amyloidosis patients from initial COVID-19 vaccine trials. Herein, we highlight many of the relevant concerns related to COVID-19 and the treatment of AL amyloidosis, summarize a general approach for AL amyloidosis management amidst the ongoing COVID-19 pandemic, and discuss current guidance about COVID-19 vaccination of patients with AL amyloidosis.
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Multiple myeloma, light chain amyloidosis, and other plasma cell dyscrasias are characterized, in part, by abnormal production of paraproteins that are often responsible for the sequelae of those diseases. These paraproteins are whole or fragmented immunoglobulins produced by clonal antibody-secreting cells (usually plasma cells, but occasionally, B lymphocytes). ⋯ Successful management of such scenarios requires a fundamental understanding of the laboratory assays at one's disposal, their role in the workup of paraproteinemias, and the interpretation thereof. This review broadly covers these assays and their roles in the diagnosis, prognosis, and management of these diseases.
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Cardiac nuclear medicine comprises various diagnostic techniques using radiopharmaceuticals for functional imaging in vivo. This article provides an overview of current clinical use of cardiac imaging in nuclear medicine in Germany: Myocardial perfusion imaging using SPECT is a well-established noninvasive tool to semi-quantitatively measure left ventricular myocardial perfusion. Ischemia and chronic myocardial scars can be idenified with a high diagnostic accuracy. ⋯ A potenitally harmful myocardial biopsy often is not needed any more and specific therapy can be initiated. In summary, diagnostic methods in cardiac nuclear medicine non-invasively allow visualization and function analysis of biological processes and are essential for diagnosis finding and therapy guidance. The continuous advancement of diagnostic tools makes nuclear cardiology a highly relevant and interesting field.
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: Anti IL-1 therapy is useful in suppressing attacks in FMF patients with colchicine resistance, however, it is not certain whether subclinical inflammation can sufficiently be inhibited with anti-IL-1 therapy in FMF patients with amyloidosis. ⋯ Anti-interleukin-1 therapy could not fully suppress the subclinical inflammatory parameters when compared to healthy individuals.
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Light chain (AL) amyloidosis is challenging to diagnose, and it should be considered a cardiac emergency. There have been a great deal of advances in the treatment of AL amyloidosis from initial descriptions of melphalan therapy until the recent approval of the first AL amyloidosis specific drug (daratumumab). ⋯ Novel antiplasma cell agents for AL like isatuximab (anti CD-38 monoclonal antibody), belantamab (anti-BCMA monoclonal antibody), and elotuzumab (anti-SLAMF7 monoclonal antibody) are currently under investigation. Both diagnostic and therapeutic advances make the future of AL management bright while acknowledging the complexity of this patient population and focusing on a multidisciplinary approach.