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Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL

Giovanni Palladini, Francesca Lavatelli, Paola Russo, Stefano Perlini, Vittorio Perfetti, Tiziana Bosoni, Laura Obici, Arthur R. Bradwell, GianVico Melzi D'Eril, Roberto Fogari, Remigio Moratti and Giampaolo Merlini

Article Figures & Data

Figures

  • Figure 1.

    Correlation between changes in NT-proBNP levels and hematologic response to chemotherapy in 51 AL patients with cardiac involvement. (A) Twenty patients in whom NT-proBNP decreased by 30% or more, or normalized; median NT-proBNP modification –52% (range: –95% to –30%). (B) Thirty-one patients in whom NT-proBNP was stable or increased, median NT-proBNP modification +37% (range: –24% to +1862%). The difference between A and B was statistically significant (P < .001) by the Mann-Whitney U test.

  • Figure 2.

    Changes in N-terminal proBNP levels in patients who reached complete remission, partial remission, or who did not respond to chemotherapy. (A) Nine patients in whom the amyloidogenic FLCs disappeared at immunofixation. (B) Thirteen patients in whom amyloidogenic FLCs decreased by more than 50%, but were still present at immunofixation. (C) Twenty-nine patients in whom FLCs remained stable or increased. The differences between A and B (P = .04), B and C (P = .001), and A and C (P < .001) were statistically significant by Mann-Whitney U test.

  • Figure 3.

    Effects of NT-proBNP and FLC reduction on survival. Effect of NT-proBNP reduction on overall (A) and progression-free (C) survival and effect of FLC reduction on overall (B) and progression-free (D) survival.

Tables

  • Table 1.

    Clinical characteristics at enrollment of 51 patients with AL and cardiac involvement

    No. (%)Median (range)
    Male 27 (53) NA
    Age, years NA 63 (28-82)
    Organ involvement
        Kidney 32 (63) NA
        Autonomic nervous system 10 (20) NA
        Liver 7 (14) NA
        Peripheral nervous system 5 (10) NA
        Soft tissues 5 (10) NA
        Gastrointestinal system 3 (6) NA
    More than one organ involved 42 (82) NA
    Serum creatinine, μM/L NA 97.2 (44.2-380.1)
    Creatinine clearance, mL/min NA 54 (15-152)
    Proteinuria, g/24 h NA 1.4 (0.1-23)
    Monoclonal component type, κ:λ 12/39 (23/77) NA
    NT-proBNP concentration, ng/L NA 2916 (393-35563)
    Mean left ventricular wall thickness, mm NA 16 (12.5-21)
    NYHA class II 22 (43) NA
    NYHA class III 29 (57) NA
    • NA indicates not applicable.

  • Table 2.

    Characteristics of 3 patients in whom mean left ventricular wall thickness decreased by 2 mm or more and NT-proBNP decreased by 30% or more after chemotherapy

    FLC, mg/LNT-proBNP, ng/LMean VWT, mmNYHA class
    Patient no.FLC typePre-TxPost-TxCRPre-TxPost-TxPre-TxPost-TxPre-TxPost-Tx
    1 λ 248.0 68.6 No 861 174 19 14 II I
    2 λ 140.0 5.3 Yes 6 097 1052 21 18 III I
    3 κ 129.0 1.7 Yes 10 447 2746 16 13 III I
    • Pre-Tx indicates before therapy; Post-Tx, after therapy; CR, complete remission at immunofixation; and VWT, ventricular-wall thickness.

  • Table 3.

    Characteristics of 4 patients who obtained 50% or greater reduction of FLC, without improvement of NT-proBNP

    FLC, mg/LNT-proBNP, ng/LMean VWT, mmNYHA class
    Patient no.FLC typePre-TxPost-TxCRPre-TxPost-TxPre-TxPost-TxPre-TxPost-Tx
    1 κ 228.0 99.1 No 1 058 1 233 14 14 II II
    2 λ 90.1 8.5 No 18 050 23 006 20 19 III III
    3 λ 173.0 63.8 No 2 706 4 046 18 20 II II
    4 λ 112.0 52.8 No 1 882 3 655 16 16 II II
    • Pre-Tx indicates before therapy; Post-Tx, after treatment; CR, complete remission at immunofixation; and VWT, ventricular-wall thickness.