This weekly series highlights eponyms in oncology. This week, we explore the history and namesake of Waldenström macroglobulinemia.
Waldenström macroglobulinemia is a lymphoma characterized by the accumulation of clonal lymphoplasmacytic cells in the bone marrow, which produce monoclonal immunoglobulin M (IgM).1
The presentation and clinical manifestations of Waldenström macroglobulinemia are caused by both tumor cell infiltration and the physicochemical and immunologic properties of the monoclonal IgM protein pentamer produced by the malignant cells.2
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Genetic testing for somatic mutation of MYD88L265P can be used to differentiate Waldenström macroglobulinemia from other conditions.1 Patient prognosis can be estimated using the International Prognostic Scoring System developed in 2009, utilizing 5 covariates — age, hemoglobin level, platelet count, beta-2-microglobulin level, and IgM concentration.3
Treatment options for Waldenström macroglobulinemia include anti-CD20 monoclonal antibody-based combinations and BTK inhibitors.1
History of the Name
Jan Gosta Waldenström, MD, is the namesake of Waldenström macroglobulinemia. Dr Waldenström was born in Stockholm in 1906.4 He obtained his medical degree at the University of Uppsala in Sweden.
After studying organic chemistry for a year in Germany, Dr Waldenström returned to the University of Uppsala and studied diseases associated with an elevation of the erythrocyte sedimentation rate (ESR).
In 1944, Dr Waldenström described 2 patients with oronasal bleeding, lymphadenopathy, anemia, increased ESR, thrombocytopenia, hypoalbuminemia, low serum fibrinogen level, and lymphoid infiltration in the bone marrow. The patients had prolonged post-operative bleeding, lobar pneumonia, and retinal hemorrhages.
The patients lacked the destructive bone lesions and bone pain that would be expected in multiple myeloma. In addition, the patients’ bone marrows were infiltrated with lymphocytes rather than plasma cells.
Both patients had poor-quality blood and bone marrow smears, and Dr Waldenström presumed the cause to be hyperproteinemia. He observed a high serum viscosity in both patients and evidence of a cryoglobulin in one of the patients.
Dr Waldenström also observed an abnormally large amount of a homogeneous globulin with a high sedimentation coefficient and an estimated molecular weight of more than 1,000,000. He theorized that this was a giant globulin molecule rather than an aggregation of smaller molecules.
This previously unrecognized condition became known as Waldenstrom macroglobulinemia.
Another of Dr Waldenström’s important contributions to medicine was the distinction between monoclonal and polyclonal gammopathies, presented in the Harvey lecture series in 1961.
After a long and productive career as a clinician and clinical investigator, Dr Waldenström died in 1996.
References
1. Dimopoulos MA, Kastritis E. How I treat Waldenstrom macroglobulinemia. Blood. 2019;134(23):2022-2035. doi:10.1182/blood.2019000725
2. Kastritis E, Leblond V, Dimopoulos MA, et al. Waldenstrom’s macroglobulinaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29(Suppl 4):iv270. doi:10.1093/annonc/mdy322
3. Morel P, Duhamel A, Gobbi P, et al. International prognostic scoring system for Waldenström macroglobulinemia. Blood. 2009;113 (18): 4163–4170. doi:https://doi.org/10.1182/blood-2008-08-174961
4. Kyle RA, Anderson KC. A tribute to Jan Gosta Waldenström. Blood. 1997;89(12):4245-7. https://doi.org/10.1182/blood.V89.12.4245
