Radiation Oncology/Waldenstroms

Waldenstrom's Macroglobulinemia

• B-cell proliferative disorder with bone marrow lymphoplasmacytic infiltration and production of monoclonal IgM; considered to be a low-grade lymphoma
• Symptoms are usually due to blood hyperviscosity, anemia, and hepatosplenomegaly
• Asymptomatic patients are typically observed
• Alkylating agents (chlorambucil), nucleoside analogues, and rituximab (eg CHOP-R) are reasonable first line approaches
• Symptomatic support as necessary
  • For RT treatment of splenomegaly, please see the Spleen chapter
  • Historically, hemibody and total body irradiation was used, but it has fallen out of favor with development of more modern chemotherapies

• Italy; 2002 PMID 12187029 -- "Advanced waldenström's macroglobulinemia: a case of possible cure after systemic chemotherapy, splenic radiation and splenectomy." (Cavanna L, Acta Haematol. 2002;108(2):97-101.)
  • Case report. 41M with WM, massive splenomegaly. Treated with plasmaparesis, chemo (BCNU, cyclophosphamide, vincristine, melphalan, prednisone) x16 cycles, and splenic RT 36 Gy
  • Outcome: Persistent mild splenomegaly and small IgM band. He then underwent splenectomy, showing persistent disease. 2 months later, complete response, and has remained disease-free x18 years
  • Conclusion: Splenectomy should be considered; spleen RT 36 Gy with chemo didn't seem sufficient to fully eradicate the disease

• Japan; 1997 PMID 9322902 -- "Durable remission after splenectomy for Waldenström's macroglobulinemia with massive splenomegaly in leukemic phase." (Takemori N, Leuk Lymphoma. 1997 Jul;26(3-4):387-93.)
  • Case report. Patient with WM and massive splenomegaly, refractory to chemo. Splenic RT effectively reduced serum IgM, spleen size, and lymphocyte counts, however, only transient
  • He then underwent splenectomy, and has been in remission for 6 years
  • Conclusion: Recommend splenectomy as part of management

• 1977 PMID 405054 -- "Report of a case of Waldenström macroglobulinemia treated and controlled by radiotherapy." (Shehata WM, Blood. 1977 Jun;49(6):1023-4.)
  • Case report

• Review; 2007 PMID 17634838 -- "Treatment of Waldenstrom's Macroglobulinemia." (Dimopoulos MA, Curr Treat Options Oncol. 2007 Apr;8(2):144-53.)