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Lymphoma

Primary systemic amyloidosis in patients with Waldenström macroglobulinemia

Leukemia volume 33, pages 790–794 (2019)Cite this article

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Waldenström macroglobulinemia (WM) is a unique IgM-associated, indolent lymphoma with at least 10% marrow lymphoplasmacytic infiltrate [1]. IgM paraprotein is implicated in 5–7% of patients with light and/or heavy chain immunoglobulin amyloidosis (AL/AHL) [2,3,4]. However, data regarding AL/AHL in WM are sparse. A greater clonal bone marrow plasma cell (BMPC) burden confers poorer survival in AL, as observed in a study from our institution (median 16 months for patients with >10% BMPCs versus 46 months), underscoring the importance of the degree of marrow infiltration at diagnosis [5]. Through this study, we attempted to address the impact of AL/AHL on the clinical course of WM and vice versa.

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References

  1. Kapoor P, Ansell SM, Fonseca R, Chanan-Khan A, Kyle RA, Kumar SK, et al. Diagnosis and Management of Waldenstrom Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines 2016. JAMA Oncol. 2017;3:1257–65.

    Article  Google Scholar 

  2. Gertz MA, Buadi FK, Hayman SR. IgM amyloidosis: clinical features in therapeutic outcomes. Clin Lymphoma Myeloma Leuk. 2011;11:146–8.

    Article  CAS  Google Scholar 

  3. Wechalekar AD, Lachmann HJ, Goodman HJ, Bradwell A, Hawkins PN, Gillmore JD. AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome. Blood. 2008;112:4009–16.

    Article  CAS  Google Scholar 

  4. Palladini G, Russo P, Bosoni T, Sarais G, Lavatelli F, Foli A, et al. AL amyloidosis associated with IgM monoclonal protein: a distinct clinical entity. Clin Lymphoma Myeloma. 2009;9:80–3.

    Article  CAS  Google Scholar 

  5. Kourelis TV, Kumar SK, Gertz MA, Lacy MQ, Buadi FK, Hayman SR, et al. Coexistent multiple myeloma or increased bone marrow plasma cells define equally high-risk populations in patients with immunoglobulin light chain amyloidosis. J Clin Oncol. 2013;31:4319–24.

    Article  CAS  Google Scholar 

  6. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, revised 4th edition, vol. 2. International Agency for Research on Cancer; 2017.

  7. Comenzo RL, Reece D, Palladini G, Seldin D, Sanchorawala V, Landau H, et al. Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis. Leukemia. 2012;26:2317–25.

    Article  CAS  Google Scholar 

  8. Sachchithanantham S, Roussel M, Palladini G, Klersy C, Mahmood S, Venner CP, et al. European Collaborative Study defining clinical profile outcomes and novel prognostic criteria in monoclonal immunoglobulin M-related light chain amyloidosis. J Clin Oncol. 2016;34:2037–45.

    Article  CAS  Google Scholar 

  9. Castillo JJ, Olszewski AJ, Kanan S, Meid K, Hunter ZR, Treon SP. Overall survival and competing risks of death in patients with Waldenstrom macroglobulinaemia: an analysis of the Surveillance, Epidemiology and End Results database. Br J Haematol. 2015;169:81–9.

    Article  Google Scholar 

  10. Muchtar E, Gertz MA, Kumar SK, Lacy MQ, Dingli D, Buadi FK, et al. Improved outcomes for newly diagnosed AL amyloidosis over the years 2000–2014: cracking the glass ceiling of early death. Blood. 2017;129:2111–19.

    Article  CAS  Google Scholar 

  11. Terrier B, Jaccard A, Harousseau JL, Delarue R, Tournilhac O, Hunault-Berger M, et al. The clinical spectrum of IgM-related amyloidosis: a French nationwide retrospective study of 72 patients. Medicine. 2008;87:99–109.

    Article  CAS  Google Scholar 

  12. Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012;30:989–95.

    Article  CAS  Google Scholar 

  13. Weiss BM, Hebreo J, Cordaro DV, Roschewski MJ, Baker TP, Abbott KC, et al. Increased serum free light chains precede the presentation of immunoglobulin light chain amyloidosis. J Clin Oncol. 2014;32:2699–704.

    Article  Google Scholar 

  14. Rajkumar SV, Kyle RA, Therneau TM, Melton LJ 3rd, Bradwell AR, Clark RJ, et al. Serum free light chain ratio is an independent risk factor for progression in monoclonal gammopathy of undetermined significance. Blood. 2005;106:812–7.

    Article  CAS  Google Scholar 

  15. Charafeddine KM, Jabbour MN, Kadi RH, Daher RT. Extended use of serum free light chain as a biomarker in lymphoproliferative disorders: a comprehensive review. Am J Clin Pathol. 2012;137:890–7.

    Article  CAS  Google Scholar 

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Acknowledgments

The contribution of S.S. and M.A.G. to this study was supported, in part, by the grants from the International Waldenström Macroglobulinemia Foundation and the Amyloidosis Foundation.

Author contributions

S.Z., J.P.A. and P.K. designed the study. S.Z., J.P.A. and P.K. collected analyzed and interpreted the data and wrote the first draft of the manuscript. S.M.A., A.D., M.A.G., E.M., S.S., N.T., S.V.R., D.D., R.G., M.Q.L., T.K., T.E.W., D.I., F.B., W.G., T.H., P.J., G.N., R.A. and S.K. interpreted the data, critically revised the manuscript and all authors approved the final version.

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Authors and Affiliations

  1. Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

    Saurabh Zanwar, Jithma P. Abeykoon, Stephen M. Ansell, Morie A. Gertz, Angela Dispenzieri, Eli Muchtar, Surbhi Sidana, Nidhi Tandon, S. Vincent Rajkumar, David Dingli, Ronald Go, Martha Q. Lacy, Taxiarchis Kourelis, Thomas E. Witzig, David Inwards, Francis Buadi, Wilson Gonsalves, Thomas Habermann, Patrick Johnston, Grzegorz Nowakowski, Robert A. Kyle, Shaji Kumar & Prashant Kapoor

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  1. Saurabh Zanwar
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  23. Prashant Kapoor
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Corresponding author

Correspondence to Prashant Kapoor.

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Conflict of interest

Dr. Ansell has received research funding from Bristol-Myers Squibb, Celldex, Merck, and Seattle Genetics. Dr. Dispenzieri has received research funding from Prothena, Jannsen, Pfizer, GSK, Takeda, Alnylam, Celgene, and serves on the advisory board for Takeda and Intellia. Dr. Gertz has received funding from Amgen, Prothena, Annexon, Appellis, Johnson and Johnson, and Celgene. Dr. Kumar has received research grants for clinical trials from Celgene, Takeda, Janssen, BMS, Sanofi, KITE, Merck, Abbvie, Medimmune, Novartis, Roche-Genentech, Amgen. Dr. Leung serves on the advisory board for Takeda and Prothena. Dr. Kapoor is principal investigator on studies for which Mayo Clinic receives funding from Takeda, Sanofi, and Amgen. The remaining authors declare that they have no conflicts of interest.

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Zanwar, S., Abeykoon, J.P., Ansell, S.M. et al. Primary systemic amyloidosis in patients with Waldenström macroglobulinemia. Leukemia 33, 790–794 (2019). https://doi.org/10.1038/s41375-018-0286-7

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