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Editorials
amyloidosis should be multidisciplinary, choice of treat- ment should be risk-adapted, and the treatment plan should be frequently verified and reconsidered based on evaluation of hematologic and organ responses.2 For these reasons, patients with AL amyloidosis should be referred to special- ized centers whenever possible.
We are now able to prognosticate survival easily and accurately in patients with AL amyloidosis both at diagno- sis and at relapse. However, there is still room for improve- ment. For instance, it is possible that more advanced renal dysfunction has a greater impact on the performance of the staging systems than a reduction of eGFR below 50 mL/min/1.73 m2. It is likely that patients with end-stage renal failure need a different approach for stratification of survival, possibly based on BNP rather than on NT-proBNP. Moreover, it is uncertain whether dFLC is the best possible marker for assessing the likelihood of late relapse, particu- larly given the availability of novel, powerful treatments. Staging systems incorporating other markers of clonal dis- ease, for instance bone marrow plasma cell infiltration or chromosomal abnormalities, should be tested for their abil- ity to predict relapse and long-term survival. Finally, the novel advanced imaging tools for the evaluation of amyloid cardiac involvement (e.g. cardiac magnetic resonance, assessment of longitudinal strain at echocardiography, positron electron tomography-computed tomography with amyloid-specific tracers) should be tested for their ability to add prognostic information to the existing staging system. International collaborative studies are the ideal setting for answering these questions quickly and reliably.
References
1. Dittrich T, Benner A, Kimmich C, et al. Performance analysis of AL amyloidosis cardiac biomarker staging systems with special focus on renal failure and atrial arrhythmia. Haematologica. 2019;104(7):1451- 1459.
2. Merlini G, Dispenzieri A, Sanchorawala V, et al. Systemic immunoglobulin light chain amyloidosis. Nat Rev Dis Primers. 2018;4(1):38.
3. MerliniG,LousadaI,AndoY,etal.Rationale,applicationandclinical qualification for NT-proBNP as a surrogate end point in pivotal clini- cal trials in patients with AL amyloidosis. Leukemia. 2016;30(10):1979-1986.
4. Palladini G, Campana C, Klersy C, et al. Serum N-terminal pro-brain
natriuretic peptide is a sensitive marker of myocardial dysfunction in
AL amyloidosis. Circulation. 2003;107(19):2440-2445.
5. AljamaMA,SidiqiMH,DispenzieriA,etal.Comparisonofdifferent techniques to identify cardiac involvement in immunoglobulin light
chain (AL) amyloidosis. Blood Adv. 2019;3(8):1226-1229.
6. Palladini G, Lavatelli F, Russo P, et al. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL.
Blood. 2006;107(10):3854-3858.
7. PalladiniG,DispenzieriA,GertzMA,etal.Newcriteriaforresponse
to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on sur- vival outcomes. J Clin Oncol. 2012;30(36):4541-4549.
8. Palladini G, Foli A, Milani P, et al. Best use of cardiac biomarkers in patients with AL amyloidosis and renal failure. Am J Hematol. 2012;87(5):465-471.
9. Dispenzieri A, Kyle R, Gertz M, et al. Survival in patients with pri- mary systemic amyloidosis and raised serum cardiac troponins. Lancet. 2003;361(9371):1787-1789.
10. Gertz MA, Lacy MQ, Dispenzieri A, et al. Refinement in patient selection to reduce treatment-related mortality from autologous stem cell transplantation in amyloidosis. Bone Marrow Transplant. 2013;48(4):557-561.
11. DispenzieriA,GertzM,KyleR,etal.SerumcardiactroponinsandN- terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004;22(18):3751-3757.
12. Wechalekar AD, Schonland SO, Kastritis E, et al. A European collab- orative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood. 2013;121(17):3420-3427.
13. PalladiniG,SachchithananthamS,MilaniP,etal.AEuropeancollab- orative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis. Blood. 2015;126(5):612-615.
14. Kumar S, Dispenzieri A, Lacy MQ, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012;30(9):989-995.
15. Lilleness B, Ruberg FL, Mussinelli R, Doros G, Sanchorawala V. Development and validation of a survival staging system incorporat- ing BNP in patients with light chain amyloidosis. Blood. 2019;133(3):215-223.
16. PalladiniG,MilaniP,FoliA,etal.Oralmelphalananddexamethasone grants extended survival with minimal toxicity in AL amyloidosis: long-term results of a risk-adapted approach. Haematologica. 2014;99(4):743-750.
17. Kumar SK, Gertz MA, Dispenzieri A. Validation of mayo clinic stag- ing system for light chain amyloidosis with high-sensitivity troponin. J Clin Oncol. 2019;37(2):171-173.
18. Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-2332.
19. Muchtar E, Therneau TM, Larson DR, et al. Comparative analysis of staging systems in AL amyloidosis. Leukemia. 2019;33(3):811-814.
20. HwaYL,GertzMA,KumarSK,etal.Prognosticrestagingatthetime of second-line therapy in patients with AL amyloidosis. Leukemia. 2019;33(5):1268-1272.
haematologica | 2019; 104(7)