Primary Amyloidosis With Renal Involvement: Outcomes in 77 Consecutive Patients at a Single Center

Wong, Sandy W.; Toskic, Denis; Warner, Melissa; Varga, Cindy; Moreno-Koehler, Alejandro; Fein, Daniel; Fogaren, Teresa; Lee, Lisa; Oliver, Colin; Guthrie, Spencer; Comenzo, Raymond L.

doi:10.1016/j.clml.2017.06.004

Cited by 13 publications

(12 citation statements)

References 42 publications

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“…Our findings are in line with prior reports from our group as well as others, which demonstrate that patients with very small light chain burden (dFLC <50 mg/l) or low light chain burden (dFLC <196 mg/l) are more likely to have renal involvement. (Kumar et al , ; Dittrich et al , ; Milani et al , ; Sidana et al , ) In another series of 77 systemic AL amyloid patients with renal involvement, Wong et al () observed median dFLC levels 127 mg/l, with 60% of patients in this series having co‐existing cardiac involvement. Similarly, Kastritis et al () described a series of 125 patients with renal involvement, of whom 42% had co‐existing cardiac involvement.…”

Section: Discussionmentioning

confidence: 67%

Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

et al. 2019

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Summary This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10%, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5‐year OS in patients undergoing transplant was 90% vs. 75% vs. 64% (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.

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Section: Discussionmentioning

confidence: 67%

Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

et al. 2019

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“…It is important to point out that ESRD rates have varied considerably among different cohorts in recent studies. The ESRD rate was 15% in the Italian cohort and 31% in the German cohort of the Palladini et al study, 11 whereas the incidence of ESRD was 33% in the studies by Kastritis et al 13 and by Wong et al 10 A study from the UK reported a dialysis progression rate of 24%. 6 The rate of ESRD in our cohort was 10%, so was more consistent with the event rate in the cohort in which the Palladini staging was derived (Italian cohort) than the Kastritis staging.…”

Section: Discussionmentioning

confidence: 96%

“…While overall survival is primarily associated with cardiac involvement in AL patients, end stage renal disease (ESRD) is determined by renal involvement 3‐6 . Studies have found hematologic response measured by the degree and rate of free light chain (FLC) reduction is associated with overall and renal survival but renal progression can occur despite patient survival 4,6‐11 . Therefore, it is important to develop criteria to assess both hematologic and renal progression and response.…”

Section: Introductionmentioning

confidence: 99%

“…[3][4][5][6] Studies have found hematologic response measured by the degree and rate of free light chain (FLC) reduction is associated with overall and renal survival but renal progression can occur despite patient survival. 4,[6][7][8][9][10][11] Therefore, it is important to develop criteria to assess both hematologic and renal progression and response.…”

Section: Introductionmentioning

confidence: 99%

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Comparison of the current renal staging, progression and response criteria to predict renal survival in AL amyloidosis using a Mayo cohort

et al. 2021

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Three sets of criteria (International Society of Amyloidosis [ISA], Palladini and Kastritis) were independently developed for staging, progression and response criteria to predict renal survival in patients with AL amyloidosis. We evaluated these criteria using a cohort of 495 newly diagnosed AL amyloidosis patients with renal involvement using time to event competing risk analysis at baseline, 3, 6 and 12 months after treatment. Only Palladini and Kastritis had a staging system and both predicted a higher risk of end stage renal disease (ESRD) in the stage III vs stage I patients but only the Palladini model was predictive for stage II patients. At 3 months, risk of ESRD was significantly higher for Palladini and ISA renal progression (hazard ratio [HR] 2.8 [95% CI: 1.5–5.3, p = .001] and 2.5 [CI: 1.4–4.6, p = .004, respectively]), but renal response was not significantly protective; conversely, the risk of ESRD was not significantly higher for the Kastritis renal progression, but was significantly protective for the Kastritis renal responders (HR 0.38 [95% CI: 0.17–0.84], p = .017). Both progression and response with ISA, Palladini and Kastritis criteria were predictive of ESRD at 6 months and 12 months. While the Palladini staging criteria at baseline, and the ISA and Palladini criteria for progression at 3 months performed better than the Kastritis criteria at baseline and 3 months post‐treatment, the Kastritis criteria performed better for response 3 months after treatment. All three sets of criteria performed well at and after 6 months post‐treatment. These differences are important when choosing endpoints for clinical trials.

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“…Dentro de las patologías sistémicas a tener en consideración, la amiloidosis cobra gran importancia dado que una detección y tratamiento precoz cambian el pronóstico de los pacientes que la padecen. En la literatura se describe que los pacientes con esta enfermedad presentan una sobrevida promedio entre 6-15 meses, usualmente falleciendo a causa del compromiso renal, falla cardíaca o neuropatía autonómica [10][11][12] .…”

Section: Discussionunclassified

Amiloidosis AL y mieloma múltiple: Macroglosia como primera manifestación de una combinación infrecuente

2020

Rev. Otorrinolaringol. Cir. Cabeza Cuello

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Primary Amyloidosis With Renal Involvement: Outcomes in 77 Consecutive Patients at a Single Center

Cited by 13 publications

References 42 publications

Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

Comparison of the current renal staging, progression and response criteria to predict renal survival in AL amyloidosis using a Mayo cohort

Amiloidosis AL y mieloma múltiple: Macroglosia como primera manifestación de una combinación infrecuente

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