Diagnosis of a difficult to differentiate case of early-onset hyperviscosity syndrome caused by IgM type multiple myelom
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CASE REPORT
Diagnosis of a difficult to differentiate case of early‑onset hyperviscosity syndrome caused by IgM type multiple myeloma: a case report Takuro Yoshimura1 · Yoshiki Hayashi1 · Katsujun Shimizu1 · Naoko Yagi1 · Minako Tsutsumi1 · Yosuke Nakaya1 · Hoyuri Fuseya1 · Mirei Horiuchi1 · Masahiro Yoshida1 · Takafumi Nakao1 · Takeshi Inoue2 · Takahisa Yamane1 Received: 6 February 2020 / Revised: 18 May 2020 / Accepted: 4 June 2020 © Japanese Society of Hematology 2020
Abstract 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. Keywords Hyperviscosity syndrome · IgM multiple myeloma · Autologous hematopoietic stem cell transplantation · Carfilzomib · Multiparameter flow cytometry
Introduction Hyperviscosity syndrome (HVS) can cause multiple organ damage if not treated immediately [1]. In general, Waldenström macroglobulinemia (WM) patients who developed symptomatic HVS were more likely to be younger than 65 years of age (78% vs. 59%) [2]. However, the frequency of its occurrence is unknown with each causative disease, and a differentiation method has not been established. IgM multiple myeloma (IgM MM) is one causative disorder underlying some cases of HVS. It is an extremely rare disease, accounting for only 0.5% of all myelomas [3, 4]. Consequently, little research has been invested in the treatment and prognosis of this disease. * Takuro Yoshimura [email protected] 1
Department of Hematology, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka, Osaka 534‑0021, Japan
Department of Pathology, Osaka City General Hospital, Osaka, Japan
2
Differentiating IgM MM from WM, which has a relatively favorable prognosis compared to IgM MM, poses a difficult challenge. The International Myeloma Working Group (IMWG) consensus panel, and the International Workshop WM (IWWM) diagnostic criteria are based on clinical symptoms such as anemia, HVS, lymphadenopathy, and systemic B symptoms, which cannot distinguish the two diseases [5–8]. Therefore, the examination of bone marrow biopsy, whole-body imaging, flow cytometry, and assessment of the presence of MyD88 gene mutation, and/ or the t(11;14) transl
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