Skip to main content

Advertisement

SpringerLink
Log in

Waldenström macroglobulinemia

  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Waldenström macroglobulinemia (WM) is a low-grade lymphoproliferative disorder characterized by the presence of an immunoglobulin M monoclonal protein in the blood and monoclonal small lymphocytes and lymphoplasmacytoid cells in the marrow. The disease is uncommon and there is a lack of clear diagnostic criteria. WM is treatable but not curable and long-term survival is possible. Therefore, the treating physician needs to carefully balance the risks and benefits of treatment. Treatments are aimed at relieving symptoms resulting from marrow infiltration and the hyperviscosity syndrome. Therapies available for initiation of treatment include alkylating agents, purine nucleoside analogs, and rituximab. Chlorambucil has been the mainstay of treatment for many years and remains useful, especially in older patients. Rituximab has become an important new therapy for this disease because of its positive treatment responses, acceptable toxicity, and lack of therapyassociated myelosuppression and myelodysplasia. Currently, rituximab is being combined with chemotherapy. Other options of treatment include interferon and corticosteroids. Emerging therapies include stem cell transplantation (autologous and allogeneic) for younger patients. Currently, there are few comparative data on which to state an absolute opinion concerning the best available treatment for patients with WM.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Waldenström J: Incipient myelomatosis or “essential” hyperglobulinemia with fibrinogenopenia: a new syndrome? Acta Med Scand 1944, 117:216–222.

    Article  Google Scholar 

  2. Harris NL, Jaffe ES, Diebold J, et al.: World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Clin Oncol 1999, 17:3835–3849.

    PubMed  CAS  Google Scholar 

  3. Wagner S, Martinelli V, Luzzato L: Similar patterns of somatic hypermutation in hairy cell leukemia, prolymphocytic leukemia, Waldenström’s macroglobulinemia and myeloma. Blood 1994, 83:3647–3653.

    PubMed  CAS  Google Scholar 

  4. Dimopoulos MA, Panayiotidis P, Moulopoulos LA, et al.: Waldenström’s macroglobulinemia: clinical features, complications, and management. J Clin Oncol 2000, 18:214–226.

    PubMed  CAS  Google Scholar 

  5. Garcia-Sanz, R., Montoto S, Torrequebrada A, et al.: Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. Br J Haematol 2001, 115:575–582. This is a report of 217 patients presenting with WM in Spain over 10 years. It presents the clinical features, the prognostic factors, the treatments received, and the causes of death of these patients.

    Article  PubMed  CAS  Google Scholar 

  6. Andriko JA, Aguilera NS, Chu WS, et al.: Waldenström’s macroglobulinemia: a clinicopathologic study of 22 cases. Cancer 1997, 80:1926–1935.

    Article  PubMed  CAS  Google Scholar 

  7. Pangalis G, Angelopoulou M, Vassilakopoulos P: Bchronic lymphocytic leukemia, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma, including Waldenström’s macroglobulinemia: a clinical, morphologic, and biologic spectrum of similar disorders. Semin Hematol 1999, 36:104–114.

    PubMed  CAS  Google Scholar 

  8. Kyle RA, Garton JP: The spectrum of IgM monoclonal gammopathy in 430 cases. Mayo Clin Proc 1987, 62:719–731.

    PubMed  CAS  Google Scholar 

  9. Kyle RA, Therneau TM, Rajkumar SV, et al.: A long-term study of prognosis in monoclonal gammopathy of undetermined significance. N Engl J Med 2002, 346:564–569. Monoclonal gammopathy of undetermined significance progressed in 115 of 1384 patients to MM, IgM lymphoma, primary amyloidosis, macroglobulinemia, chronic lymphocytic leukemia, or plasmacytoma. This is the largest follow-up study of patients with MGUS.

    Article  PubMed  Google Scholar 

  10. Gertz MA, Kyle RA: Hyperviscosity syndrome. J Intensive Care 1995, 10:128–141.

    CAS  Google Scholar 

  11. Kyle RA, Greipp PR, Gertz MA, et al.: Waldenström’s macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil. Br J Haematol 2000, 108:737–742. Seventy-nine percent of 24 patients administered continuous therapy had an objective improvement by reduction of serum M protein or increase in hemoglobin. Sixty-eight percent of 22 patients administered chlorambucil intermittently had an objective response. This is one of the most important studies showing effectiveness of chlorambucil in patients with WM.

    Article  PubMed  CAS  Google Scholar 

  12. Case DC Jr, Ervin TJ, Boyd MA, et al.: Waldenström’s macroglobulinemia: long-term results with the M-2 protocol. Cancer Invest 1991, 9:1–7.

    PubMed  Google Scholar 

  13. Laurencet FM, Zulian GB, Guetty-Alberto M, et al.: Cladribine with cyclophosphamide and prednisone in the management of low-grade lymphoproliferative malignancies. Br J Cancer 1999, 79:1215–1219.

    Article  PubMed  CAS  Google Scholar 

  14. Dhodapkar MV, Jacobson JL, Gertz MA, et al.: Prognostic factors and response to fludarabine therapy in patients with Waldenström macroglobulinemia: results of United States intergroup trial (Southwest Oncology Group S9003). Blood 2001, 98:41–48. One hundred eighty-two patients with symptomatic or progressive disease were treated with four to eight cycles of therapy with fludarabine. The overall rate of response to therapy was 36%, with 2% complete remission. Patients who were 70 years or older had a substantially lower likelihood of response.

    Article  PubMed  CAS  Google Scholar 

  15. Dimopoulos MA, Kantarjian H, Estey E, et al.: Treatment of Waldenström macroglobulinemia with 2-chlorodeoxyadenosine. Ann Intern Med 1993, 118:195–198.

    PubMed  CAS  Google Scholar 

  16. Byrd JC, White CA, Link B, et al.: Rituximab therapy in Waldenström’s macroglobulinemia: preliminary evidence of clinical activity. Ann Oncol 1999, 10:1525–1527.

    Article  PubMed  CAS  Google Scholar 

  17. Treon SP, Agus DB, Link B, et al.: CD20-directed antibody-mediated immunotherapy induces responses and facilitates hematologic recovery in patients with Waldenström’s macroglobulinemia. J Immunother 2001, 24:272–279.

    Article  CAS  Google Scholar 

  18. Dimopoulos MA, Zervas C, Zomas A, et al.: Treatment of Waldenström’s macroglobulinemia with rituximab. J Clin Oncol 2002, 20:2327–2333. This is a prospective phase II study to define the activity of rituximab in patients with WM. Responses occurred in 40% of previously untreated patients and 50% of pretreated patients.

    Article  PubMed  CAS  Google Scholar 

  19. Treon SP, Anderson KC: The use of rituximab in the treatment of malignant and nonmalignant plasma cell disorders. Semin Oncol 2000, 27(Suppl 12):79–85.

    PubMed  CAS  Google Scholar 

  20. Witzig TE, Gordon LI, Cabanillas F, et al.: Randomized controlled trial of yttrium-90-labeled ibritumomab tiuxetan radioimmunotherapy versus rituximab immunotherapy for patients with relapsed or refractory lowgrade, follicular, or transformed B-cell non-Hodgkin’s lymphoma. J Clin Oncol 2002, 20:2453–2463.

    Article  PubMed  CAS  Google Scholar 

  21. Kapff C, Jandl J: Blood: Atlas and Sourcebook of Hematology. Boston: Little Brown; 1991.

    Google Scholar 

  22. Dutcher T, Fahey J: The histopathology of macroglobulinemia of Waldenström. J Natl Cancer Inst 1959, 22:887–917.

    PubMed  CAS  Google Scholar 

  23. Bartl R, Frisch B, Mahl G, et al.: Bone marrow histology in Waldenström’s macroglobulinaemia. Clinical relevance of subtype recognition. Scand J Haematol 1983, 31:359–375.

    Article  PubMed  CAS  Google Scholar 

  24. Owen R, Johnson S, Morgan G: Waldenström’s macroglobulinemia: laboratory diagnosis and treatment. Hematol Oncol 2000, 18:41–49.

    Article  PubMed  CAS  Google Scholar 

  25. Owen RG, Barrans SL, Richards SJ, et al.: Waldenström macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors. Am J Clin Pathol 2001, 116:420–428.

    Article  PubMed  CAS  Google Scholar 

  26. Kucharska-Pulczynska M, Ellegard J, Hokland P: Analysis of leukocyte differentiation antigens in blood and bone marrow from patients with Waldenström macroglobulinemia. Br J Haematol 1987, 65:395–399.

    PubMed  CAS  Google Scholar 

  27. Feiner H, Rizk C, Finfer M: IgM monoclonal gammopathy/ Waldenström’s macroglobulinemia: a morphological and immunophenotypic study of the bone marrow. Mod Pathol 1990, 3:348–356.

    PubMed  CAS  Google Scholar 

  28. Nagai M, Ikeda K, Nakamura H, et al.: Splenectomy for a case with Waldenström macroglobulinemia with giant splenomegaly. Am J Hematol 1991, 37:140.

    Article  PubMed  CAS  Google Scholar 

  29. Humphrey JS, Conley CL: Durable complete remission of macroglobulinemia after splenectomy: a report of two cases and review of the literature. Am J Hematol 1995, 48:262–266.

    Article  PubMed  CAS  Google Scholar 

  30. Winter AJ, Obeid D, Jones EL: Long survival after splenic immunoblastic transformation of Waldenström’s macroglobulinaemia. Br J Haematol 1995, 91:412–414.

    PubMed  CAS  Google Scholar 

  31. Dimopoulos MA, Zomas A, Viniou NA, et al.: Treatment of Waldenström’s macroglobulinemia with thalidomide. J Clin Oncol 2001, 19:3595–3601.

    Google Scholar 

  32. Desikan R, Dhodapkar M, Siegel D, et al.: High-dose therapy with autologous haemopoietic stem cell support for Waldenström’s macroglobulinaemia. Br J Haematol 1999, 105:993–996.

    Article  PubMed  CAS  Google Scholar 

  33. Dreger P, Glass B, Kuse R, et al.: Myeloablative radiochemotherapy followed by reinfusion of purged autologous stem cells for Waldenström’s macroglobulinaemia. Br J Haematol 1999, 106:115–118.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematology, Mayo Clinic, 200 First Street, 55905, Rochester, MN, USA

    Thomas E. Witzig MD

Authors
  1. Irene M. Ghobrial MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Thomas E. Witzig MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ghobrial, I.M., Witzig, T.E. Waldenström macroglobulinemia. Curr. Treat. Options in Oncol. 5, 239–247 (2004). https://doi.org/10.1007/s11864-004-0015-5

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11864-004-0015-5

Keywords

Search

Navigation