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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2020; 41(04): 596-601
DOI: 10.4103/ijmpo.ijmpo_234_19
Case Report

Waldenström Macroglobulinemia: A Clinicopathological Profile and Review of Six Cases

Priyanka Samal
Department of Clinical Hematology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India
,
Prantar Chakrabarti
Department of Clinical Hematology, Nilratan Sircar Medical College, Kolkata, West Bengal, India
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Abstract

Waldenström macroglobulinemia (WM) is an indolent B-cell neoplasm in which lymphoplasmacytic cells are accumulated in the bone marrow. This rare disease is incurable with the available treatments and its management is generally based on risk-adapted methods. We present here six cases of WM with their clinicopathological profile and response to therapy. This is a retrospective observational study conducted in a tertiary referral center in Eastern India from October 2014 to December 2016. The clinical manifestations, diagnostic challenges, and response to therapy for WM were analyzed. A total of six cases of WM were analyzed, of which five were male; the mean age was 65 years. The most common presentation was transfusion-dependent anemia, followed by hyperviscosity. All the patients had bone marrow infiltration by lymphoplasmacytoid cells, and serum protein electrophoresis and immunofixation showed immunoglobulin M kappa monoclonal protein. Bortezomib, dexamethasone, and rituximab (BDR) was the most commonly used regimen. Complete and very good partial responses were seen in one patient (16.7%) each and partial response in 3 (50%) patients. There was disease progression to diffuse large B-cell lymphoma in one patient (16.7%). After a 26-month follow-up, four of six patients (66.67%) remained free of disease progression. A very low incidence of WM was reported, and patients had a varied clinicopathological spectrum. A combination of BDR is a promising frontline therapy in the patients of WM.

Keywords

Bortezomib - BDR regimen - rituximab - Waldenström macroglobulinemia


Publication History

Received: 16 November 2019

Accepted: 31 January 2020

Article published online:
17 May 2021

© 2020. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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  • References

  • 1 Fonseca R, Witzig TE. Waldenström macroglobulinemia. In: Greer JP, Forester J, Lukens JN, Rodgers GM, Paraskevas F, Glader B. editors Wintrobe's Clinical Hematology. Philadelphia: Lippincott Williams and Wilkins; 2004: 266774
    Google Scholar
  • 2 Herrinton LJ, Weiss NS. Incidence of Waldenstrom's macroglobulinemia. Blood 1993; 82: 3148-50
    CrossrefPubMedGoogle Scholar
  • 3 Dimopoulos MA, Galani E, Matsouka C. Waldenström's macroglobulinemia. Hematol Oncol Clin North Am 1999; 13: 1351-66
    CrossrefPubMedGoogle Scholar
  • 4 Treon SP. How I treat Waldenström macroglobulinemia. Blood 2015; 126: 721-32
    CrossrefPubMedGoogle Scholar
  • 5 Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML. et al. Clinicopathological definition of Waldenstrom's macroglobulinemia: Consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. Semin Oncol 2003; 30: 110-5
    CrossrefPubMedGoogle Scholar
  • 6 Owen RG, Kyle RA, Stone MJ, Rawstron AC, Leblond V, Merlini G. et al. Response assessment in Waldenström macroglobulinaemia: Update from the VIth International Workshop. Br J Haematol 2013; 160: 171-6
    CrossrefPubMedGoogle Scholar
  • 7 Vijay A, Gertz MA. Waldenström macroglobulinemia. Blood 2007; 109: 5096-103
    CrossrefPubMedGoogle Scholar
  • 8 Buske C, Leblond V. How to manage Waldenstrom's macroglobulinemia. Leukemia 2013; 27: 762-72
    CrossrefPubMedGoogle Scholar
  • 9 Björkholm M, Johansson E, Papamichael D, Celsing F, Matthews J, Lister TA. et al. Patterns of clinical presentation, treatment, and outcome in patients with Waldenstrom's macroglobulinemia: A two-institution study. Semin Oncol 2003; 30: 226-30
    CrossrefPubMedGoogle Scholar
  • 10 Oza A, Rajkumar SV. Waldenstrom macroglobulinemia: Prognosis and management. Blood Cancer J 2015; 5: e394
    CrossrefPubMedGoogle Scholar
  • 11 Gustine JN, Meid K, Dubeau T, Hunter ZR, Xu L, Yang G. et al. Serum IgM level as predictor of symptomatic hyperviscosity in patients with Waldenström macroglobulinaemia. Br J Haematol 2017; 177: 717-25
    CrossrefPubMedGoogle Scholar
  • 12 Ansell SM, Kyle RA, Reeder CB, Fonseca R, Mikhael JR, Morice WG. et al. Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. Mayo Clin Proc 2010; 85: 824-33
    CrossrefPubMedGoogle Scholar
  • 13 Lin P, Mansoor A, Bueso-Ramos C, Hao S, Lai R, Medeiros LJ. Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Clinicopathologic features of 12 cases. Am J Clin Pathol 2003; 120: 246-53
    CrossrefPubMedGoogle Scholar
  • 14 Gertz MA. Waldenström macroglobulinemia: 2012 update on diagnosis, risk stratification, and management. Am J Hematol 2012; 87: 503-10
    CrossrefPubMedGoogle Scholar
  • 15 Dimopoulos MA, Weber DM, Kantarjian H, Keating M, Alexanian R. 2Chlorodeoxyadenosine therapy of patients with Waldenstrom macroglobulinemia previously treated with fludarabine. Ann Oncol 1994; 5: 288-9
    CrossrefPubMedGoogle Scholar
  • 16 Weide R, Heymanns J, Köppler H. Induction of complete haematological remission after monotherapy with anti-CD20 monoclonal antibody (RITUXIMAB) in a patient with alkylating agent resistant Waldenström's macroglobulinaemia. Leuk Lymphoma 1999; 36: 203-6
    CrossrefPubMedGoogle Scholar
  • 17 Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Zervas K, Tsatalas C, Kokkinis G. et al. Primary treatment of Waldenström macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol 2007; 25: 3344-9
    CrossrefPubMedGoogle Scholar
  • 18 Gavriatopoulou M, García-Sanz R, Kastritis E, Morel P, Kyrtsonis MC, Michalis E. et al. BDR in newly diagnosed patients with WM: Final analysis of a phase 2 study after a minimum follow-up of 6 years. Blood 2017; 129: 456-9
    CrossrefPubMedGoogle Scholar
  • 19 Treon SP, Ioakimidis L, Soumerai JD, Patterson CJ, Sheehy P, Nelson M. et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180. J Clin Oncol 2009; 27: 3830-5
    CrossrefPubMedGoogle Scholar
  • 20 Dimopoulos MA, García-Sanz R, Gavriatopoulou M, Morel P, Kyrtsonis MC, Michalis E. et al. 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: 3276-82
    CrossrefPubMedGoogle Scholar
  • 21 Benevolo G, LobettiBodoni C, Orsucci L, Botto B, Chiappella A, Riccomagno P. et al. In: Safety and Efficacy of Bendamustine Therapy in Waldenström Macroglobulinemia. Amsterdam: Poster Session Presented at the 17th Congress of European Hematology Association; 2012
  • 22 Treon SP, Hanzis C, Tripsas C, Ioakimidis L, Patterson CJ, Manning RJ. et al. Bendamustine therapy in patients with relapsed or refractory Waldenström's macroglobulinemia. Clin Lymphoma Myeloma Leuk 2011; 11: 133-5
    CrossrefPubMedGoogle Scholar
  • 23 Abeykoon JP, Zanwar S, Ansell SM, Kumar S, Thompson CA, Habermann TM. Outcomes with rituximab plus bendamustine (R-Benda), dexamethasone, rituximab, cyclophosphamide (DRC), and bortezomib, dexamethasone, rituximab (BDR) as primary therapy in patients with Waldenstrom macroglobulinemia (WM). J Clin Oncol 2019; 37: 7509 et al Suppl 15
    CrossrefGoogle Scholar