Waldenström macroglobulinemia, previously also known as lymphoplasmacytic lymphoma, is a type of B-cell lymphoma.
Recent publications classify Waldenström macroglobulinemia as a lymphoplasmacytic lymphoma with any level of an IgM paraprotein 10. Therefore the two entities are not synonymous.
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.
Treatment and prognosis
The International Prognostic Scoring System for Waldenström's 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.
- Bing-Neel syndrome: malignant lymphocyte infiltration into the central nervous system (CNS) 11
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
- 2. Cheson BD (2006). "Chronic Lymphoid Leukemias and Plasma Cell Disorders". In Dale DD, Federman DD. ACP Medicine. New York, NY: WebMD Professional Publishing. ISBN 0-9748327-1-5.
- 3. Leleu X, Gay J, Roccaro AM et-al. Update on therapeutic options in Waldenström macroglobulinemia. Eur. J. Haematol. 2009;82 (1): 1-12. Eur. J. Haematol. (full text) - doi:10.1111/j.1600-0609.2008.01171.x - Free text at pubmed - Pubmed citation
- 4. Dimopoulos MA, Panayiotidis P, Moulopoulos LA et-al. Waldenström's macroglobulinemia: clinical features, complications, and management. J. Clin. Oncol. 2000;18 (1): 214-26. J. Clin. Oncol. (full text) - Pubmed citation
- 5. Moulopoulos LA, Dimopoulos MA, Varma DG et-al. Waldenström macroglobulinemia: MR imaging of the spine and CT of the abdomen and pelvis. Radiology. 1993;188 (3): 669-73. Radiology (abstract) - Pubmed citation
- 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 MC, 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 citation
- 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 SP. How I treat Waldenström macroglobulinemia. Blood. 2009;114 (12): 2375-85. Blood (full text) - doi:10.1182/blood-2009-05-174359 - Pubmed citation
- 10. Lin P, Molina TJ, Cook JR et-al. 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 citation
- 11. Kim HJ, Suh SI, Kim JH, Kim BJ. Brain magnetic resolution imaging to diagnose Bing-Neel syndrome. (2009) Journal of Korean Neurosurgical Society. 46 (6): 588-91. doi:10.3340/jkns.2009.46.6.588 - Pubmed
- 12. Ribatti D. Protagonists of Medicine. (2010) ISBN: 9789048137411
- 13. Richard Thomas, Marta Braschi-Amirfarzan, Shawn L. Laferriere, Jyothi P. Jagannathan. Imaging of Waldenström Macroglobulinemia: A Comprehensive Review for the Radiologist in the Era of Personalized Medicine. (2019) American Journal of Roentgenology. doi:10.2214/AJR.19.21493 - Pubmed
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