Hyperviscosity syndrome (HVS) can cause multiple organ damage if not treated immediately. IgM multiple myeloma (IgM MM) is a very rare form of myeloma with clinical features such as elevated serum IgM, and anemia, that resemble Waldenström macroglobulinemia (WM). Distinguishing between these two diseases is important, but can be a challenging problem. It is well known that MyD88 mutations and t(11;14) translocations are useful for differential diagnosis. We diagnosed HVS in a 29-year-old male with IgM MM. He was treated with triplet therapy, autologous hematopoietic stem cell transplantation, and carfilzomib consolidation therapy. His clinical course was monitored by serum IgM levels, and bone marrow myeloma cell counts by multiparameter flow cytometry analysis. After this series of treatments, his HSV disappeared and he reached stringent complete response. In cases of early onset of HVS, IgM MM should be considered in addition to WM.
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Gertz MA. Acute hyperviscosity: syndromes and management. Blood. 2018;132(13):1379–85.
Gustine JN, Meid K, Dubeau T, Hunter ZR, Xu L, Yang G, et al. Serum IgM level as predictor of symptomatic hyperviscosity in patients with Waldenström macroglobulinaemia. Br J Haematol. 2017;177(5):717–25.
Kyle RA, Gertz MA, Witzig TE, Lust JA, Lacy MQ, Dispenzieri A, et al. Review of 1027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc. 2003;78(1):21–33.
Castillo JJ, Jurczyszyn A, Brozova L, Crusoe E, Czepiel J, Davila J, et al. IgM myeloma: a multicenter retrospective study of 134 patients. Am J Hematol. 2017;92(8):746–51.
Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15(12):e538–48.
Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, et al. Clinicopathological definition of Waldenstrom’s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom’s Macroglobulinemia. Semin Oncol. 2003;30(2):110–5.
Dimopoulos MA, Panayiotidis P, Moulopoulos LA, Sfikakis P, Dalakas M. Waldenström’s macroglobulinemia: clinical features, complications, and management. J Clin Oncol. 2000;18(1):214–26.
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.
Avet-Loiseau H, Garand R, Lodé L, Harousseau JL, Bataille R. Translocation t(11;14)(q13;q32) is the hallmark of IgM, IgE, and nonsecretory multiple myeloma variants. Blood. 2003;101(4):1570–1.
Xu L, Hunter ZR, Yang G, Zhou Y, Cao Y, Liu X, et al. MyD88 L265P in Waldenström macroglobulinemia, immunoglobulin M monoclonal gammopathy, and other B-cell lymphoproliferative disorders using conventional and quantitative allele-specific polymerase chain reaction. Blood. 2013;121(11):2051–8.
Attal M, Lauwers-Cances V, Hulin C, Leleu X, Caillot D, Escoffre M, et al. Lenalidomide, bortezomib and dexamethasone with transplantation for myeloma. N Eng J Med. 2017;376(14):1311–20.
Schuster SR, Rajkumar SV, Dispenzieri A, Morice W, Aspitia AM, Ansell S, et al. IgM multiple myeloma: disease definition, prognosis, and differentiation from Waldenström’s macroglobulinemia. Am J Hematol. 2010;85(11):853–5.
Treon SP. How I treat Waldenström macroglobulinemia. Blood. 2015;126(6):721–32.
Kumar S, Paiva B, Anderson KC, Durie B, Landgren O, Moreau P, et al. International Myeloma Working Group consensus criteria for response and minimal residual disease assessment in multiple myeloma. Lancet Oncol. 2016;17(8):e328–46.
Romano A, Palumbo GA, Parrinello NL, Conticello C, Martello M, Terragna C, et al. Minimal residual disease assessment within the bone marrow of multiple myeloma: a review of caveats, clinical significance and future perspectives. Front Oncol. 2019;9:699.
Munshi NC, Avet-Loiseau H, Rawstron AC, Owen RG, Child JA, Thakurta A, et al. Association of minimal residual disease with superior survival outcomes in patients with multiple myeloma: a meta-analysis. JAMA Oncol. 2017;3(1):28–35.
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Yoshimura, T., Hayashi, Y., Shimizu, K. et al. Diagnosis of a difficult to differentiate case of early-onset hyperviscosity syndrome caused by IgM type multiple myeloma: a case report. Int J Hematol (2020). https://doi.org/10.1007/s12185-020-02917-7
- Hyperviscosity syndrome
- IgM multiple myeloma
- Autologous hematopoietic stem cell transplantation
- Multiparameter flow cytometry