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Maria Stella Graziani

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Martina Zaninotto

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Bruna Lo Sasso
Giampaolo Merlini
Martina Montagnana
Andrea Mosca
Paola Pezzati
Rossella Tomaiuolo
Matteo Vidali

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Sergio Bernardini Italy
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Philippe Gillery France
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Magdalena Krintus Poland
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☩Howard Morris Australia
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Giuseppe Agosta

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Arianna Lucini Paioni
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email: biochimica.clinica@sibioc.it



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BC: Articoli scritti da M. Vidus Rosin

Identificazione di danno renale reversibile e di precoce risposta alla chemioterapia in pazienti con amiloidosi AL
Identification of reversible renal damage and early response to chemotherapy in AL amyloidosis
<p>The kidney&nbsp;is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or&nbsp;reversibility of renal involvement. Furthermore, criteria for renal response have never been validated. We designed a&nbsp;staging system for renal damage and identified criteria for renal response and progression in a population of 732&nbsp;newly diagnosed patients with AL amyloidosis. The population was composed of 461 patients from Pavia (testing&nbsp;cohort) and 271 subjects from Heidelberg (validation cohort). Baseline proteinuria &gt;5 g/24 h and estimated glomerular&nbsp;filtration rate (eGFR) &lt;50 mL/min/1.73 m<sup>2</sup> were independently associated with poorer renal survival and discriminated&nbsp;between 3 stages (with none, one or two markers above the cut-off) with significant different renal survival. At 6-month&nbsp;follow-up, a &ge;25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal&nbsp;progression. A decrease in proteinuria &ge;30% or below the cut-off of 0.5 g/24 h in absence of renal progression were&nbsp;the criteria for renal response, being associated with longer renal survival in the testing and validation cohorts. These&nbsp;endpoints can be used as validated response criteria in renal AL amyloidosis, allowing early assessment of treatment&nbsp;efficacy.</p>
Biochimica Clinica ; 40(1) 21-27
Contributi scientifici - Scientific Papers
 
Amiloidosi AL: il cuore del problema
AL amyloidosis: the heart of the problem
<p>Immunoglobulin light chain amyloidosis (AL) is characterized by the&nbsp;production of immunoglobulin light chains with conformational abnormalities that cause systemic toxicity with rapid&nbsp;deterioration of the function of vital organs. When the heart is involved, as it is the case in ~3/4 of patients, clinical signs&nbsp;and symptoms often appear when organ damage is already irreversible and the treatment cannot longer change the&nbsp;course of disease. Although in recent years new powerful therapeutic regimens have become available, which are able&nbsp;to significantly improve long-term survival, the mortality rate in the first year after diagnosis has indeed not improved, still&nbsp;being 25-30%. Cardiac involvement is responsible for almost all of these deaths. Early diagnosis based on biochemical&nbsp;markers of the disease rather than on clinical symptoms and signs can allow for early detection of patients with cardiac&nbsp;amyloidosis and to establish an effective therapy. To this end, our group has proposed the introduction of the&nbsp;measurement of natriuretic peptides that can identify the presence of amyloid cardiomyopathy with a sensitivity of 100%&nbsp;in the monitoring of subjects with monoclonal gammopathies of undetermined significance (MGUS) and altered ratio of&nbsp;circulating free light chains (FLC). Individuals with MGUS and altered FLC ratio are at intermediate/high risk of&nbsp;developing a malignant disease (AL amyloidosis in 10-15% of cases) and, according to the guidelines of the International&nbsp;Myeloma Working Group, they should be monitored regularly for their entire life. Here we describe a case where the&nbsp;application of these recent recommendations has allowed the timely recognition of amyloid cardiomyopathy.</p>
Biochimica Clinica ; 39(3) 220-222
Casi clinici - Case report