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Waldenström’s Macroglobulinemia pp 265–275Cite as

Response Assessment in Waldenström’s Macroglobulinaemia

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Abstract

Formal criteria for assessment of treatment response for patients with Waldenström’s macroglobulinaemia (WM) were first proposed by a consensus panel during the Second International Workshop on WM in 2003 (Weber D, Treon SP, Emmanouilides C, Branagan AR, Byrd JC, Bladé J, Kimby E. Uniform response criteria in Waldenstrom’s macroglobulinaemia: consensus panel recommendations from the Second International Workshop on Waldenstrom’s Macroglobulinaemia. Semin Oncol 2003;30:127–131). New biological knowledge and new treatment paradigms have made updates necessary, both for clinicians and authorities, especially for evaluation, approval and remuneration of new drugs. The most updated consensus panel response definitions include evaluation of the monoclonal immunoglobulin M in serum, the tumour burden in bone marrow and in extramedullary sites and symptoms of disease. The value of quantitative assessment of the tumour in bone marrow, imaging techniques for extramedullary/nodal disease and assessment of haematological response is still under discussion.

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References

  1. Owen RG, Treon SP, Al-Katib A, et al. Clinicopathological definition of Waldenstrom’s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom’sMacroglobulinemia. Semin Oncol. 2003;30(2):110–5.

    Article  PubMed  Google Scholar 

  2. Swerdlow SH. WHO classification of tumours of haematopoietic and lymphoid tissues, vol. 2. Geneva: World Health Organization; 2008.

    Google Scholar 

  3. Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, Sheehy P, Manning RJ, Patterson CJ, Tripsas C, Arcaini L, Pinkus GS, Rodig SJ, Sohani AR, Harris NL, Laramie JM, Skifter DA, Lincoln SE, Hunter ZR. MYD88 L265P somatic mutation in Waldenström’s macroglobulinemia. N Engl J Med. 2012;367(9):826–33.

    Article  CAS  PubMed  Google Scholar 

  4. Weber D, Treon SP, Emmanouilides C, Branagan AR, Byrd JC, Bladé J, Kimby E. Uniform response criteria in Waldenstrom’s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom’s Macroglobulinemia. Semin Oncol. 2003;30:127–31.

    Article  PubMed  Google Scholar 

  5. Kimby E, Treon SP, Anagnostopoulos A, Dimopoulos M, Garcia-Sanz R, Gertz MA, Johnson S, LeBlond V, Fermand JP, Maloney DG, Merlini G, Morel P, Morra E, Nichols G, Ocio EM, Owen R, Stone M, Bladé J. Update on recommendations for assessing response from the Third International Workshop on Waldenstrom’s Macroglobulinemia. Clin Lymphoma Myeloma. 2006;6(5):380–3.

    Article  PubMed  Google Scholar 

  6. Owen RG, Kyle RA, Stone MJ, Rawstron AC, Leblond V, Merlini G, Garcia-Sanz R, Ocio EM, Morra E, Morel P, Anderson KC, Patterson CJ, Munshi NC, Tedeschi A, Joshua DE, Kastritis E, Terpos E, Ghobrial IM, Leleu X, Gertz MA, Ansell SM, Morice WG, Kimby E, Treon SP. Response assessment in Waldenström macroglobulinaemia: update from the VIth International Workshop. Br J Haematol. 2013;160(2):171–6.

    Article  PubMed  Google Scholar 

  7. Cheson BD, Fisher RI, Barrington SF, Cavalli F, Schwartz LH, Zucca E, Lister TA. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059–68.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J, Crowley J, Ocio EM, Garcia-Sanz R, Treon SP, Leblond V, Kyle RA, Barlogie B, Merlini G. International prognostic scoring system for Waldenstrom macroglobulinemia. Blood. 2009;113(18):4163–70.

    Article  CAS  PubMed  Google Scholar 

  9. Gertz MA, Abonour R, Heffner LT, Greipp PR, Uno H, Rajkumar SV. Clinical value of minor responses after 4 doses of rituximab in Waldenström macroglobulinaemia: a follow-up of the Eastern Cooperative Oncology Group E3A98 trial. Br J Haematol. 2009;147:677–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Treon SP, Yang G, Hanzis C, Ioakimidis L, Verselis SJ, Fox EA, Xu L, Hunter ZR, Tseng H, Manning RJ, Patterson CJ, Sheehy P, Turnbull B. Attainment of complete/very good partial response following rituximab-based therapy is an important determinant to progression-free survival, and is impacted by polymorphisms in FCGR3A in Waldenstrom macroglobulinaemia. Br J Haematol. 2011;154(2):223–8.

    Article  PubMed  Google Scholar 

  11. Treon SP, Xu L, Hunter Z, et al. MYD88 mutations and response to ibrutinib in Waldenström’s macroglobulinemia. N Engl J Med. 2015;373:584–6.

    Article  CAS  PubMed  Google Scholar 

  12. Hunter ZR, Manning RJ, Hanzis C, Ciccarelli BT, Ioakimidis L, Patterson CJ, Lewicki MC, Tseng H, Gong P, Liu X, Zhou Y, Yang G, Sun J, Xu L, Sheehy P, Morra M, Treon SP. IgA and IgG hypogammaglobulinemia in Waldenström’s macroglobulinemia. Haematologica. 2010;95(3):470–5.

    Article  CAS  PubMed  Google Scholar 

  13. García-Sanz R, Ocio E, Caballero A, Magalhães RJP, Alonso J, López-Anglada L, Villaescusa T, Puig N, Hernández JM, Fernández-Calvo J, Aguilar A, Martín A, López R, Paiva B, Orfao A, Vidriales B, San-Miguel JF, Del Carpio D. Post-treatment bone marrow residual disease > 5% by flow cytometry is highly predictive of short progression-free and overall survival in patients with Waldenström’s macroglobulinemia. Clin Lymphoma Myeloma Leuk. 2011;11:168.

    Article  PubMed  Google Scholar 

  14. Böttcher S, Ritgen M, Kneba M. Flow cytometric MRD detection in selected mature B-cell malignancies. Methods Mol Biol. 2013;971:149–74.

    Article  PubMed  Google Scholar 

  15. Rawstron AC, Böttcher S, Letestu R, Villamor N, Fazi C, Kartsios H, de Tute RM, Shingles J, Ritgen M, Moreno C, Lin K, Pettitt AR, Kneba M, Montserrat E, Cymbalista F, Hallek M, Hillmen P, Ghia P. European research initiative in CLL Improving efficiency and sensitivity: European research initiative in CLL (ERIC) update on the international harmonised approach for flow cytometric residual disease monitoring in CLL. Leukemia. 2013;27(1):142–9.

    Article  CAS  PubMed  Google Scholar 

  16. Kastritis E, Kyrtsonis MC, Morel P, Gavriatopoulou M, Hatjiharissi E, Symeonidis AS, Vassou A, Repousis P, Delimpasi S, Sioni A, Michalis E, Michael M, Vervessou E, Voulgarelis M, Tsatalas C, Terpos E, Dimopoulos MA. Competing risk survival analysis in patients with symptomatic Waldenström macroglobulinemia: the impact of disease unrelated mortality and of rituximab-based primary therapy. Haematologica. 2015;100(11):e446–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Castillo JJ, Olszewski AJ, Kanan S, Meid K, Hunter ZR, Treon SP. Overall survival and competing risks of death in patients with Waldenström macroglobulinaemia: an analysis of the surveillance, epidemiology and end results database. Br J Haematol. 2015;169(1):81–9.

    Article  PubMed  Google Scholar 

  18. Riches PG, Sheldon J, Smith AM, Hobbs JR. Overestimation of monoclonal immunoglobulin by immunochemical methods. Ann Clin Biochem. 1991;28(Pt 3):253–9.

    Article  PubMed  Google Scholar 

  19. Murray DL, Ryu Snyder MR, Katzmann JA. Quantitation of serum monoclonal proteins: relationship between agarose gel electrophoresis and immunonephelometry. Clin Chem. 2009;55(8):1523–9.

    Article  CAS  PubMed  Google Scholar 

  20. Del Giudice I, Matutes E, Osuji N, Parry-Jones N, Swansbury J, Catovsky D. Delayed response to fludarabine in lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia. Haematologica. 2005;90:268–70.

    PubMed  Google Scholar 

  21. Varghese AM, Rawstron AC, Ashcroft AJ, Moreton P, Owen RG. Assessment of bone marrow response in Waldenström’s macroglobulinemia. Clin Lymphoma Myeloma. 2009;9:53–5.

    Article  PubMed  Google Scholar 

  22. Tedeschi A, Benevolo G, Varettoni M, Battista ML, Zinzani PL, Visco C, Meneghini V, Pioltelli P, Sacchi S, Ricci F, Nichelatti M, Zaja F, Lazzarino M, Vitolo U, Morra E. Fludarabine plus cyclophosphamide and rituximab in Waldenstrom macroglobulinemia: an effective but myelosuppressive regimen to be offered to patients with advanced disease. Cancer. 2012;118(2):434–43.

    Article  CAS  PubMed  Google Scholar 

  23. Chen CI, Kouroukis CT, White D, Voralia M, Stadtmauer E, Stewart AK, Wright JJ, Powers J, Walsh W, Eisenhauer E, National Cancer Institute of Canada Clinical Trials Group. Bortezomib is active in patients with untreated or relapsed Waldenstrom’s macroglobulinemia: a phase II study of the National Cancer Institute of Canada Clinical Trials Group. J Clin Oncol. 2007;25(12):1570–5.

    Article  CAS  PubMed  Google Scholar 

  24. Treon SP, Ioakimidis L, Soumerai JD, Patterson CJ, Sheehy P, Nelson M, et al. Primary therapy of Waldenströmmacroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05–180. J Clin Oncol. 2009;27:3830–35.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Dimopoulos MA, García-Sanz R, Gavriatopoulou M, Morel P, Kyrtsonis MC, Michalis E, Kartasis Z, Leleu X, Palladini G, Tedeschi A, Gika D, Merlini G, Kastritis E, Sonneveld P. Primary therapy of Waldenstrom macroglobulinemia (WM) with weekly bortezomib, low-dose dexamethasone, and rituximab (BDR): long-term results of a phase 2 study of the European Myeloma Network (EMN). Blood. 2013;122(19):3276–82.

    Article  CAS  PubMed  Google Scholar 

  26. Ghobrial IM, Fonseca R, Greipp PR, Blood E, Rue M, Vesole DH, Gertz MA, Eastern Cooperative Oncology Group. Initial immunoglobulin M’flare’ after rituximab therapy in patients diagnosed with Waldenstrom macroglobulinemia: an eastern cooperative oncology group study. Cancer. 2004;101(11):2593–8.

    Article  CAS  PubMed  Google Scholar 

  27. Barakat FH, Medeiros LJ, Wei EX, Konoplev S, Lin P, Jorgensen JL. Residual monotypic plasma cells in patients with Waldenstrom macroglobulinemia after therapy. Am J Clin Pathol. 2011;135:365–73.

    Article  PubMed  Google Scholar 

  28. Treon SP, Hunter ZR, Matous J, Joyce RM, Mannion B, Advani R, Cook D, Songer J, Hill J, Kaden BR, Sharon D, Steiss R, Leleu X, Branagan AR, Badros A. Multicenter clinical trial of bortezomib in relapsed/refractory Waldenstrom’s macroglobulinemia: results of WMCTG Trial 03–248. Clin Cancer Res. 2007;13(11):3320–5.

    Article  CAS  PubMed  Google Scholar 

  29. Itzykson R, Le Garff-Tavernier M, Katsahian S, Diemert MC, Musset L, Leblond V. Serum-free light chain elevation is associated with a shorter time to treatment in Waldenstrom’s macroglobulinemia. Haematologica. 2008;93(5):793–4.

    Article  PubMed  Google Scholar 

  30. Leleu X, Moreau A-S, Weller E, Roccaro AM, Coiteux V, Manning R, Nelson M, Leduc R, Robu D, Dupire S, Hatjiharissi E, Burwick N, Darre S, Hennache B, Treon SP, Facon T, Gertz MA, Ghobrial IM. Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. Leuk Lymphoma. 2008;49:1104–7.

    Article  CAS  PubMed  Google Scholar 

  31. Leleu X, Xie W, Bagshaw M, Banwait R, Leduc R, Roper N, Weller E, Ghobrial IM. The role of serum immunoglobulin free light chain in response and progression in Waldenstrom macroglobulinemia. Clin Cancer Res. 2011;17:3013–8.

    Article  CAS  PubMed  Google Scholar 

  32. Leleu X, Koulieris E, Maltezas D, Itzykson R, Xie W, Manier S, Dulery R, Boyle E, Gauthier J, Poulain S, Tatiana T, Panayiotidis P, Bradwell AR, Harding S, Leblond V, Kyrtsonis M-C, Ghobrial IM. Novel M-component based biomarkers in Waldenström’s macroglobulinemia. Clin Lymphoma, Myeloma Leuk. 2011;11:164–7.

    Article  CAS  Google Scholar 

  33. Gnemmi V, Leleu X, Provot F, Moulonguet F, Buob D, et al. Cast nephropathy and light-chain deposition disease in Waldenström macroglobulinemia. Am J Kidney Dis. 2012;60(3):487–91.

    Article  PubMed  Google Scholar 

  34. Paiva B, Montes MC, García-Sanz R, Ocio EM, Alonso J, de Las HN, Escalante F, Cuello R, de Coca AG, Galende J, Hernández J, Sierra M, Martin A, Pardal E, Bárez A, Alonso J, Suarez L, González-López TJ, Perez JJ, Orfao A, Vidríales MB, San Miguel JF. Multiparameter flow cytometry for the identification of the Waldenström’s clone in IgM-MGUS and Waldenström’s Macroglobulinemia: new criteria for differential diagnosis and risk stratification. Leukemia. 2014;28(1):166–73.

    Article  CAS  PubMed  Google Scholar 

  35. Jiménez C, Sebastián E, Chillón MC, Giraldo P, Mariano Hernández J, Escalante F, González-López TJ, Aguilera C, de Coca AG, Murillo I, Alcoceba M, Balanzategui A, Sarasquete ME, Corral R, Marín LA, Paiva B, Ocio EM, Gutiérrez NC, González M, San Miguel JF, García-Sanz R. MYD88 L265P is a marker highly characteristic of, but not restricted to Waldenström’s macroglobulinemia. Leukemia. 2013;27(8):1722–8.

    Article  PubMed  Google Scholar 

  36. Banwait R, O’Regan K, Campigotto F, Harris B, Yarar D, Bagshaw M, Leleu X, Leduc R, Ramaiya N, Weller E, Ghobrial IM. The role of 18F-FDG PET/CT imaging in Waldenstrom macroglobulinemia. Am J Hematol. 2011;86:567–72.

    Article  PubMed  Google Scholar 

  37. Treon SP, Cao Y, Xu L, Yang G, Liu X, Hunter ZR. Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia. Blood. 2014;123(18):2791–6.

    Article  CAS  PubMed  Google Scholar 

  38. Falchi L, Keating MJ, Marom EM, Truong MT, Schlette EJ, Sargent RL, Trinh L, Wang X, Smith SC, Jain N, Estrov Z, O'Brien S, Wierda WG, Lerner S, Ferrajoli A. Correlation between FDG/PET, histology, characteristics, and survival in 332 patients with chronic lymphoid leukemia. Blood. 2014;123(18):2783–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Papajík T, Mysliveček M, Urbanová R, Buriánková E, Kapitáňová Z, Procházka V, Turcsányi P, Formánek R, Henzlová L, Flodr P, Jarošová M, Indrák K. 2-[18F]fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography examination in patients with chronic lymphocytic leukemia may reveal Richter transformation. Leuk Lymphoma. 2014;55(2):314–9.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Hematology, Department of Medicine at Huddinge, Karolinska Institute and University Hospital, Stockholm, Sweden

    Eva Kimby

  2. HMDS Laboratory, St James’s Institute of Oncology, Leeds, UK

    Roger G. Owen

  3. Niguarda Ca’Granda Hospital, 20162, Milan, Italy

    Enrica Morra

Authors
  1. Eva Kimby
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  2. Roger G. Owen
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  3. Enrica Morra
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Correspondence to Eva Kimby .

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Editors and Affiliations

  1. Haematology, Hôpital Pitié-Salpêtriêre, Paris, France

    Véronique Leblond

  2. Dana Farber Cancer Institute, Boston, Massachusetts, USA

    Steve Treon

  3. School of Medicine, University of Athens, Athens, Greece

    Meletios Dimoploulos

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Kimby, E., Owen, R.G., Morra, E. (2017). Response Assessment in Waldenström’s Macroglobulinaemia. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_18

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  • DOI: https://doi.org/10.1007/978-3-319-22584-5_18

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