Citation, DOI and article data
Recent publications classify Waldenström macroglobulinemia as a subtype of lymphoplasmacytic lymphoma with any level of an IgM paraprotein; these two conditions are not synonymous 10.
It is a rare condition, accounting for only 1% of all lymphoproliferative disorders. The incidence is 1,500 cases per annum in the United States. The median age of onset is 60-65 years. A minority of cases occur in late adolescence.
Most patients are asymptomatic at the time of diagnosis, which is often incidentally made during blood tests for an unrelated condition. If symptoms occur, the most common ones are fatigue (2/3 of cases) and polyneuropathy (1/4 of cases). Otherwise, the presentation includes the triad of non-specific B-symptoms: fever, night sweats, and unintentional weight loss. Another feature is hyperviscosity syndrome.
The pathogenesis is poorly understood but may resemble that of chronic lymphocytic leukemia. Clonal proliferation of functionally impaired B-cells occurs, as well as infiltration of various tissues (predominantly bone marrow, spleen, and lymph nodes). There is an overproduction of IgM, leading to hyperviscosity syndrome and resultant impaired microcirculation.
There have been relatively few articles on the imaging features of Waldenström macroglobulinemia. Authorities do not recommend imaging as part of the initial workup of patients 14.
Bone marrow involvement is the commonest imaging manifestation, best appreciated on MRI. One study found that MRI spines were abnormal in 91% cases with two main appearances seen 14:
- diffuse: all bone marrow signal is abnormal
- heterogeneous: myriad small foci of abnormal signal scattered throughout uninvolved bone marrow
Treatment and prognosis
The International Prognostic Scoring System for Waldenström macroglobulinemia is a predictive model for characterization of long-term outcome. Factors predicting survival are:
- age >65 years
- hemoglobin ≤11.5 g/dL
- platelet count ≤100×109/L
- B2-microglobulin >3 mg/L
- serum monoclonal protein concentration >70 g/L
Risk categories are:
- low: ≤1 adverse variable except age
- intermediate: 2 adverse characteristics or age >65 years
- high: >2 adverse characteristics
Five-year survival rates for these categories are 87%, 68% and 36% respectively.
Untreated, the disease typically progresses indolently, and behaves differently among patients.
History and etymology
This condition was first described in 1944 by Swedish heme-oncologist Jan G (Gosta) Waldenström (1906-1996) 1,12.
- 1. Waldenström JG. Incipient myelomatosis or „essential“ hyperglobulinemia with fibrinogenopenia - a new syndrome? (1944) Acta med Scand. 117:216-247
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- 4. Dimopoulos M, Panayiotidis P, Moulopoulos L, Sfikakis P, Dalakas M. Waldenström's Macroglobulinemia: Clinical Features, Complications, and Management. J Clin Oncol. 2000;18(1):214-26. doi:10.1200/JCO.2000.18.1.214 - Pubmed
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- 6. Morel P, Duhamel A, Gobbi P, Dimopoulos M, Dhodapkar M, McCoy J, et al. International Prognostic Scoring System for Waldenström’s Macroglobulinemia. XIth International Myeloma Workshop & IVth International Workshop on Waldenstrom's Macroglobulinemia 25 30 June 2007 Kos Island, Greece. Haematologica 2007;92(6 suppl 2):1-229.
- 7. Kastritis E, Kyrtsonis M, Hadjiharissi E et al. Validation of the International Prognostic Scoring System (IPSS) for Waldenstrom's Macroglobulinemia (WM) and the Importance of Serum Lactate Dehydrogenase (LDH). Leuk Res. 2010;34(10):1340-3. doi:10.1016/j.leukres.2010.04.005 - Pubmed
- 8. Ansell SM, Kyle RA, Reeder CB et-al. Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. Mayo Clin. Proc. 2010;85 (9): 824-33. doi:10.4065/mcp.2010.0304 - Free text at pubmed - Pubmed citation
- 9. Treon S. How I Treat Waldenström Macroglobulinemia. Blood. 2009;114(12):2375-85. doi:10.1182/blood-2009-05-174359 - Pubmed
- 10. Lin P, Molina T, Cook J, Swerdlow S. Lymphoplasmacytic Lymphoma and Other Non-Marginal Zone Lymphomas with Plasmacytic Differentiation. Am J Clin Pathol. 2011;136(2):195-210. doi:10.1309/AJCP8FOIVTB6LBER - Pubmed
- 11. Kim H, Suh S, Kim J, Kim B. Brain Magnetic Resolution Imaging to Diagnose Bing-Neel Syndrome. J Korean Neurosurg Soc. 2009;46(6):588-91. doi:10.3340/jkns.2009.46.6.588 - Pubmed
- 12. Domenico Ribatti. Protagonists of Medicine. (2010) ISBN: 9789048137411 - Google Books
- 13. Thomas R, Braschi-Amirfarzan M, Laferriere S, Jagannathan J. Imaging of Waldenström Macroglobulinemia: A Comprehensive Review for the Radiologist in the Era of Personalized Medicine. AJR Am J Roentgenol. 2019;213(6):W248-56. doi:10.2214/AJR.19.21493 - Pubmed
- 14. Thomas R, Braschi-Amirfarzan M, Laferriere S, Jagannathan J. Imaging of Waldenström Macroglobulinemia: A Comprehensive Review for the Radiologist in the Era of Personalized Medicine. AJR Am J Roentgenol. 2019;213(6):W248-56. doi:10.2214/ajr.19.21493 - Pubmed