PMID- 35045672 OWN - NLM STAT- MEDLINE DCOM- 20220121 LR - 20220214 IS - 0253-2727 (Print) IS - 2707-9740 (Electronic) IS - 0253-2727 (Linking) VI - 42 IP - 12 DP - 2021 Dec 14 TI - [Immunoglobulin M multiple myeloma: a six-case report and literature review]. PG - 1011-1014 LID - 10.3760/cma.j.issn.0253-2727.2021.12.008 [doi] AB - Objective: To investigate the clinical characteristics, responses, and prognosis of immunoglobulin M multiple myeloma (IgM MM) . Methods: The clinical characteristics, laboratory results, bone marrow biopsy results, response, and prognosis of six cases of IgM MM in the Blood Diseases Hospital, Chinese Academy of Medical Sciences, from December 18, 2009 to October 29, 2020 were collected and analyzed. Results: All six cases met the diagnosis criteria of IgM MM. There were four males and two females. The median age at first diagnosis was 70 (59-81) years. According to Durie-Salmon (DS) staging, 2 cases were in ⅠA, and 4 cases were in ⅢA. According to the International Staging System (ISS) , 4 cases were in Ⅱ, and 2 cases were in Ⅲ. The initial symptoms were as follows: 4 cases of bone pain, 3 cases of hyperviscosity, and 2 cases of lymphadenopathy or hepatosplenomegaly. Laboratory results showed the following: median blood M protein: 39.11 (3.61-75.56) g/L; median serum IgM: 69.35 (4.35-137.00) g/L; median hemoglobin: 87.0 (70-131) g/L; median blood creatinine: 83.6 (53.0-129.6) mumol/L; median blood calcium: 2.12 (2.11-2.50) mmol/L. The median ratio of bone marrow plasma cells was 0.390 (0.255-0.590) , and in four cases, plasma cells were observed in blood smears. Karyotype analysis and fluorescence in situ hybridization (FISH) examination showed the following: 1 case of hypodiploidy, 2 cases of P53 gene deletion, 1 case of 1q21 amplification positive, and 4 cases of RB-1 gene deletion positive. The immunoglobulin heavy chain (IgH) rearrangement was positive in all cases, of which 3 cases were CCND1/IgH fusion gene-positive identified with t (11;14) rearrangement. Immunophenotyping revealed that all cases were positive for CD38, CD138, and monoclonal light chain and four cases were weakly positive for CD20. All cases accepted proteasome inhibitor-based regimens and attained the response of partial remission to strict complete remission. Conclusion: In addition to the typical clinical manifestations of myeloma, IgM MM is also characterized by hyperviscosity, lymphadenopathy, or hepatosplenomegaly, and t (11;14) is the most frequent cytogenetics aberration. Furthermore, the response and prognosis of IgM MM are similar to other common myeloma subtypes. FAU - Yan, W W AU - Yan WW AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Fan, H S AU - Fan HS AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Xu, J Y AU - Xu JY AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Liu, J H AU - Liu JH AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Du, C X AU - Du CX AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Deng, S H AU - Deng SH AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Sui, W W AU - Sui WW AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Xu, Y AU - Xu Y AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - Qiu, L G AU - Qiu LG AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. FAU - An, G AU - An G AD - State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. LA - chi GR - 81920108006/International Cooperation and Exchanges Project of National Natural Science Foundation of China/ PT - Case Reports PT - Journal Article PT - Review PL - China TA - Zhonghua Xue Ye Xue Za Zhi JT - Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi JID - 8212398 RN - 0 (Immunoglobulin M) SB - IM MH - Female MH - Humans MH - Immunoglobulin M MH - In Situ Hybridization, Fluorescence MH - Male MH - *Multiple Myeloma/diagnosis MH - Plasma Cells MH - Prognosis PMC - PMC8770889 OTO - NOTNLM OT - Hyperviscosity OT - Immunoglobulin M OT - Multiple myeloma EDAT- 2022/01/21 06:00 MHDA- 2022/01/22 06:00 PMCR- 2021/12/01 CRDT- 2022/01/20 01:32 PHST- 2022/01/20 01:32 [entrez] PHST- 2022/01/21 06:00 [pubmed] PHST- 2022/01/22 06:00 [medline] PHST- 2021/12/01 00:00 [pmc-release] AID - cjh-42-12-1011 [pii] AID - 10.3760/cma.j.issn.0253-2727.2021.12.008 [doi] PST - ppublish SO - Zhonghua Xue Ye Xue Za Zhi. 2021 Dec 14;42(12):1011-1014. doi: 10.3760/cma.j.issn.0253-2727.2021.12.008.