Incidence rate of amyloidosis in patients from a medical care program in Buenos Aires, Argentina: a prospective cohort

Aguirre, María Adela; Boietti, Bruno; Nucifora, Elsa; Sorroche, Patricia; Quirós, Fernán González Bernaldo de; Giunta, Diego; Posadas‐Martinez, María Lourdes

doi:10.1080/13506129.2016.1207626

Cited by 23 publications

(18 citation statements)

References 14 publications

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“…These light chains form oligomers and bind to cellular and extracellular matrix proteins causing direct toxicity and amyloid deposition in organs such as the heart and kidneys 1,2 . Studies are limited regarding the annual incidence of AL, but estimates range from 3 to 13 cases per million person-years [2][3][4][5][6][7][8][9] .…”

Section: Introductionmentioning

confidence: 99%

Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis

et al. 2021

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Venetoclax is efficacious in relapsed/refractory t(11;14) multiple myeloma, thus warranting investigation in light-chain amyloidosis (AL). This retrospective cohort includes 43 patients with previously treated AL, from 14 centers in the US and Europe. Thirty-one patients harbored t(11;14), 11 did not, and one t(11;14) status was unknown. Patients received a venetoclax-containing regimen for at least one 21- or 28-day cycle; the median prior treatments was three. The hematologic response rate for all patients was 68%; 63% achieved VGPR/CR. t(11;14) patients had higher hematologic response (81% vs. 40%) and higher VGPR/CR rate (78% vs. 30%, odds ratio: 0.12, 95% CI 0.02–0.62) than non-t(11;14) patients. For the unsegregated cohort, median progression-free survival (PFS) was 31.0 months and median OS was not reached (NR). For t(11;14), median PFS was NR and for non-t(11;14) median PFS was 6.7 months (HR: 0.14, 95% CI 0.04–0.53). Multivariate analysis incorporating age, sex, prior lines of therapy, and disease stage suggested a risk reduction for progression or death in t(11;14) patients. Median OS was NR for either subgroup. The organ response rate was 38%; most responders harbored t(11;14). Grade 3 or higher adverse events occurred in 19% with 7% due to infections. These promising results require confirmation in a randomized clinical trial.

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Section: Introductionmentioning

confidence: 99%

Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis

et al. 2021

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“…One epidemiologic study reported incidence densities of 6.13 per million person-years for AL amyloidosis and 1.21 per million person-years for AA amyloidosis. 7 Depending on the type of amyloidosis, GI tract involvement varies. The GI tract is affected in approximately 60% of patients with AA amyloidosis, but less common in patients with AL amyloidosis, with only 8% of patients.…”

Section: Discussionmentioning

confidence: 99%

Colonoscopy Leading to the Diagnosis of AL Amyloidosis in the Gastrointestinal Tract Mimicking an Acute Ulcerative Colitis Flare

et al. 2019

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The 2 most common types of amyloidosis are light chain (AL) and reactive (AA). AL is associated with plasma cell dyscrasias; reactive (AA) is associated with chronic inflammatory conditions. A few cases have described AL amyloidosis mimicking colitis. However, endoscopic findings leading to the diagnosis of AL amyloidosis are rare. We report a 77-year-old woman with a medical history of ulcerative colitis who presented with recurrent nonbloody watery diarrhea. Colonoscopy revealed features suspicious for amyloidosis. Bone marrow biopsy showed multiple myeloma and AL amyloidosis. This case demonstrates the importance of generating a broad differential and the pivotal role of endoscopic findings in diagnosing uncommon diseases.

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“…The presence of organ involvement was studied: cardiac involvement (development of congestive heart failure, arrhythmia attributable to amyloidosis, or biopsy positive for amyloidosis, imaging involvement (cardiac MRI with gadolinium, echocardiogram with strain)); neurological involvement (polyneuropathy, autonomic dysfunction attributable to amyloidosis or biopsy positive for amyloidosis); nephrologic involvement (kidney failure, nephrotic syndrome, urine protein attributable to amyloidosis, or biopsy positive for amyloidosis); and involvement of other organs attributable to amyloidosis or biopsy positive for amyloidosis (Aguirre et al, 2016 ).…”

Section: Methodsmentioning

confidence: 99%

Epidemiology of variant transthyretin amyloidosis at a reference center in Argentina

Sáez

Aguirre

Arenaza

et al. 2021

Molec Gen & Gen Med

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Incidence rate of amyloidosis in patients from a medical care program in Buenos Aires, Argentina: a prospective cohort

Abstract: This is the first paper to report the incidence density of ATTRwt, AL and AA amyloidosis in Latin America. Our results are consistent with other studies from other regions. Although systemic amyloidosis is a rare disease, it is a major health problem because of its morbi-mortality.

Cited by 23 publications

References 14 publications

Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis

Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis

Colonoscopy Leading to the Diagnosis of AL Amyloidosis in the Gastrointestinal Tract Mimicking an Acute Ulcerative Colitis Flare

Epidemiology of variant transthyretin amyloidosis at a reference center in Argentina

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