Immunoglobulin M multiple myeloma: a six-case report and literature review
10.3760/cma.j.issn.0253-2727.2021.12.008
- VernacularTitle:IgM型多发性骨髓瘤六例报告并文献复习
- Author:
Wenqiang YAN
1
;
Huishou FAN
;
Jingyu XU
;
Jiahui LIU
;
Chenxing DU
;
Shuhui DENG
;
Weiwei SUI
;
Yan XU
;
Lugui QIU
;
Gang AN
Author Information
1. 中国医学科学院血液病医院（中国医学科学院血液学研究所），实验血液学国家重点实验室，国家血液系统疾病临床医学研究中心，天津 300020
- Keywords:
Multiple myeloma;
Immunoglobulin M;
Hyperviscosity
- From:
Chinese Journal of Hematology
2021;42(12):1011-1014
- CountryChina
- Language:Chinese
-
Abstract:
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) μmol/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.
