Comparison of Free Light Chain Assays

Mahmood, Shameem; Wassef, Nancy; Salter, Simon J; Sachchithanantham, Sajitha; Lane, Thirusha; Foard, Darren; Whelan, Carol; Lachmann, HJ; Gillmore, Julian D.; Hawkins, Philip N.; Wechalekar, Ashutosh

doi:10.1093/ajcp/aqw079

Cited by 7 publications

(4 citation statements)

References 17 publications

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“…Therefore, Freelite cut‐off values are not equivalent to N‐Latex assay values for AL staging. So clinicians should consider using the same assay for follow‐up 34 …”

Section: Discussionmentioning

confidence: 99%

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Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Zaroui,

Kharoubi,

Gounot

et al. 2024

ESC Heart Failure

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AimsPredicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage.Methods and resultsThis prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision‐making regarding therapy. Over the 12‐year study period, among the 233 patients included, 133 were NYHA III‐IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT‐proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut‐off values for Stage 1: hsTnT ≤ 107 ng/L and NT‐proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT‐proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT‐proBNP then CB 4 μmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 μmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2–24]. At 1 year, 102 (44%) patients died and the Kaplan–Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11–18]) and stage 3, 6.6 months (95% CI [1–13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2.ConclusionsThe Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis.

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“…Therefore, Freelite cut‐off values are not equivalent to N‐Latex assay values for AL staging. So clinicians should consider using the same assay for follow‐up 34 …”

Section: Discussionmentioning

confidence: 99%

“…So clinicians should consider using the same assay for follow-up. 34 Although both testing systems are now used in diagnostic laboratories, the reference values and clinical cut-offs are specific, 17,35 but clinical cut-offs for N-Latex are still needed for AL-CA prognostic testing.…”

Section: Measuring Direct Free Light Chains With N-latex Technique Ma...mentioning

confidence: 99%

Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Zaroui,

Kharoubi,

Gounot

et al. 2024

ESC Heart Failure

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“…Currently, hematologic response criteria are validated only for serum FLCs measured by the Freelite assay 45 ; new assays are available but have not been validated and FLC measurements are not interchangeable. 46 , 47 Reduction in the concentration of the FLC levels is the strongest predictor for prolonged survival. Even among patients with low baseline FLC levels (below “measurable” threshold), further reduction improves organ function and survival.…”

Section: Response Assessmentmentioning

confidence: 99%

“…Clonal response assessment is based on serum FLCs which may be, however, below the level of reliable quantification in ∼20% of patients. Currently, hematologic response criteria are validated only for serum FLCs measured by the Freelite assay 45 ; new assays are available but have not been validated and FLC measurements are not interchangeable 46,47 . Reduction in the concentration of the FLC levels is the strongest predictor for prolonged survival.…”

Section: Response Assessmentmentioning

confidence: 99%

Systemic AL Amyloidosis: Current Approaches to Diagnosis and Management

Fotiou

Dimopoulos

2020

HemaSphere

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AL amyloidosis is characterized by a low-level expansion of an indolent, small plasma cell clone that produces amyloidogenic light chains. Amyloid aggregates or preceding intermediaries cause direct cell damage through their proteotoxicity, and amyloid deposits distort tissue architecture, and, eventually, lead to organ impairment. It is a rare, underdiagnosed disease with a diverse clinical presentation depending on the organ tropism of the amyloid fibrils; cardiac and renal involvement is most common, but any organ can be affected, excluding the central nervous system. A high level of awareness and a systematic approach using newly emerging screening biomarkers is required to achieve early diagnosis. Management should be multidisciplinary as supportive management tailored to management of organ dysfunction is paramount to survival and minimization of treatment-associated toxicity. The initial therapeutic aim is to rapidly eliminate the clonal plasma cell that produces the circulating amyloid precursor and achieve a complete hematologic response, and if possible with undetectable minimal residual disease as assessed by next-generation methods (flow and sequencing), with minimal toxicity. Treatment is tailored to the initial risk assessment of the patients. Treatments are based on regimens adapted from the expanding options that are available for multiple myeloma patients and hematological response rates have improved. Organ response rates are strongly associated with deeper hematologic response but usually lag behind hematological response and are also dependent on the initial organ function reserve. Agents directed against the amyloid deposits have been explored to aid amyloid clearance and improve organ function, but data are still negative.

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