News in AL Amyloidosis ASH 2016

Agis, Hermine

doi:10.1007/s12254-017-0332-6

Cited by 4 publications

(4 citation statements)

References 39 publications

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“…The endoscopic findings have been reported to be very diverse but characteristic [1]. The treatment for AL amyloidosis with evidence of organ involvement is chemotherapy and autologous stem cell transplantation [2]. In our case, although the clinical course at the first episode was compatible with ischemic colitis, retrospective evaluation of biopsy specimens from the first episode revealed they were positive for Congo red staining.…”

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confidence: 72%

Kappa light-chain amyloidosis of gastrointestinal tract

Hashimoto

Matsuda

2018

aog

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confidence: 72%

Kappa light-chain amyloidosis of gastrointestinal tract

Hashimoto

Matsuda

2018

aog

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“…Amyloidosis was diagnosed by performing biopsy on periumbelical fat (109), rectum mucosa (7), heart (2), liver (7) and kidney (10). All patients underwent ultrasound evaluation of the heart, an electrocardiogram and laboratory tests to characterize the disease.…”

Section: Methodsmentioning

confidence: 99%

“…Symptoms and signs are mostly non-specific, which is a further obstacle to making an early [10,11] diagnosis, complicated by the high prevalence of other cardiovascular diseases such as coronary heart disease, arrhythmias, and valvular heart diseases. In cases of a high clinical suspicion of a restrictive etiology of unexplained cardiomyopathy is essential.…”

Section: Introductionmentioning

confidence: 99%

Right Heart Changes Impact on Clinical Phenotype of Amyloid Cardiac Involvement: A Single Centre Study

Cicco

Solimando

Buono

et al. 2020

Life

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Amyloidosis is due to deposition of an excessive amount of protein in many parenchymal tissues, including myocardium. The onset of cardiac Amyloidosis (CA) is an inauspicious prognostic factor, which can lead to sudden death. We retrospectively analyzed 135 patients with systemic amyloidosis, admitted to our ward between 1981 and 2019. Among them, 54 patients (46.30% F/53.70% M, aged 63.95 ± 12.82) presented CA at baseline. In 53 patients, it was associated with a multiorgan involvement, while in one there was a primary myocardial deposition. As a control group, we enrolled 81 patients (49.30% F/50.70% M, aged 58.33 ± 15.65) who did not meet the criteria for CA. In 44/54 of patients CA was associated with AL, 5/54 with AA and 3/54 of patients with ATTR, and in 1/54 AL was related to hemodialysis and in 1/54 to Gel-Amyloidosis. The most common AL type was IgG (28/44); less frequent forms were either IgA (7/44) or IgD (2/44), while seven patients had a λ free light chain form. The 32 AL with complete Ig were 31 λ-chain and just one k-chain. CA patients presented normal BP (SBP 118.0 ± 8.4 mmHg; DBP 73.8 ± 4.9 mmHg), while those with nCA had an increased proteinuria (p = 0.02). TnI and NT-proBNP were significantly increased compared to nCA (p = 0.031 and p = 0.047, respectively). In CA patients we found an increased LDH compared to nCA (p = 0.0011). CA patients were also found to have an increased interventricular septum thickness compared to nCA (p = 0.002), a decreased Ejection Fraction % (p = 0.0018) and Doppler velocity E/e’ ratio (p = 0.0095). Moreover, CA patients had an enhanced right atrium area (p = 0.0179), right ventricle basal diameter (p = 0.0112) and wall thickness (p = 0.0471) compared to nCA, and an increased inferior cava vein diameter (p = 0.0495) as well. TAPSE was the method chosen to evaluate systolic function of the right heart. In CA subjects very poor TAPSE levels were found compared to nCA patients (p = 0.0495). Additionally, we found a significant positive correlation between TAPSE and lymphocyte count (r = 0.47; p = 0.031) as well as Gamma globulins (r = 0.43, p = 0.033), Monoclonal components (r = 0.72; p = 0.047) and IgG values (r = 0.62, p = 0.018). Conversely, a significant negative correlation with LDH (r = −0.57, p = 0.005), IVS (r = −0.51, p = 0.008) and diastolic function evaluated as E/e’ (r = −0.60, p = 0.003) were verified. CA patients had very poor survival rates compared to controls (30 vs. 66 months in CA vs. nCA, respectively, p = 0.15). Mean survival of CA individuals was worse also when stratified according to NT-proBNP levels, using 2500 pg/mL as class boundary (174 vs. 5.5 months, for patients with lower vs. higher values than the median, respectively p = 0.013). In much the same way, a decreased right heart systolic function was correlated with a worse prognosis (18.0 months median survival, not reached in subjects with lower values than 18 mm, p = 0.0186). Finally, our data highlight the potential prognostic and predictive value of right heart alterations characterizing amyloidosis, as a novel clinical parameter correlated to increased LDH and immunoglobulins levels. Overall, we confirm the clinical relevance of cardiac involvement suggests that right heart evaluation may be considered as a new marker for clinical risk stratification in patients with amyloidosis.

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“…However, the importance of having critical knowledge about when to suspect AL amyloidosis and how to achieve a correct diagnosis has been increasingly recognized. Hermine Agis is reviewing the recent developments as presented at the ASH meeting, as well as giving important background information on the disease [3]. Therapeutic progress in myelodysplastic syndromes seems to be an ongoing struggle, but potential molecular targets are now increasingly N. Zojer ( ) 1st…”

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confidence: 99%

Notes from California – experts review the ASH meeting 2016 in San Diego

Zojer

2017

memo

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Again MEMO has asked experts in the field to review their choice of presentations from the ASH meeting 2016, held in San Diego from the 3 rd to the 6 th December last year. Reviews on selected topics in hematology were invited, reflecting dynamic advancements in understanding and treatment of hematologic disorders. Exciting times we live in as hematologists! Daniel Heintel reviews novel strategies in the management of lymphomas and readers will find important, practice-changing information coming from large phase III trials like the GALLIUM and LyMA trials in his contribution [1]. Arnold Bolomsky and Heinz Ludwig cover the area relating to treatment of high-risk myeloma [2]. At the 2016 ASH meeting, already close to 15% of myeloma abstracts dealt with this difficult-to-treat subset of myeloma patients. In the contribution, readers will find results of groundbreaking studies presented at the meeting and information on a series of new substances, for which early results raise hope that significant progress can be achieved in the area.AL amyloidosis has long been a disease, which due to its rarity, has been in the focus of only a few specialists. However, the importance of having critical knowledge about when to suspect AL amyloidosis and how to achieve a correct diagnosis has been increasingly recognized. Hermine Agis is reviewing the recent developments as presented at the ASH meeting, as well as giving important background information on the disease [3]. Therapeutic progress in myelodysplastic syndromes seems to be an ongoing struggle, but potential molecular targets are now increasingly N. Zojer ( ) 1st

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News in AL Amyloidosis ASH 2016

Cited by 4 publications

References 39 publications

Kappa light-chain amyloidosis of gastrointestinal tract

Kappa light-chain amyloidosis of gastrointestinal tract

Right Heart Changes Impact on Clinical Phenotype of Amyloid Cardiac Involvement: A Single Centre Study

Notes from California – experts review the ASH meeting 2016 in San Diego

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