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目的:探讨IgD型多发性骨髓瘤(MM)的临床特点及治疗方案,从而提高对IgD型MM的诊疗水平。方法:回顾性分析17例初诊IgD型MM患者的临床资料特点、预后分期及不同的治疗方案对生存的影响。结果:同期284例初诊MM患者中,有17例为IgD型MM患者,占6.0%。发病时中位年龄为51(33~77)岁,男∶女为1.43∶1.00。DS分期中Ⅲ期14例(82.4%),其中B期6例(35.3%);ISS分期中Ⅲ期10例(58.8%)。IgD轻链以λ常见(16例),占94.1%。血、尿单克隆轻链异常增高,而血清总球蛋白及IgG、IgA、IgM含量偏低或正常。初诊时骨痛及骨质破坏分别占41.2%和47.1%,贫血占70.6%,肾功能损害占47.1%。13例骨髓检查发现异常的浆细胞增生。髓外浸润(经病理证实)发生率占58.8%,其中3例患者存在胸椎或腰椎椎体旁软组织包块,且2例发生截瘫;7例患者有胸腔积液;1例患者胸骨后占位;1例甲状腺及乳腺占位。6/13例采用硼替佐米为基础的化疗方案,其生存曲线呈现优于传统化疗的趋势,但差异无统计学意义(P=0.212)。结论:IgD型MM发病率低,但侵袭性高,应常规进行免疫固定电泳检测IgD以免漏诊和误诊,采用蛋白酶体抑制剂等新药联合治疗可能有益于延长患者的生存。
Objective: To investigate the clinical features and treatment of IgD multiple myeloma (MM) so as to improve the diagnosis and treatment of IgD type MM. Methods: A retrospective analysis of 17 cases of newly diagnosed IgD type MM patients with clinical data characteristics, prognosis staging and different treatment options on the impact of survival. Results: Of 284 newly diagnosed MM patients in the same period, 17 were IgD type MM patients, accounting for 6.0%. The median age at onset was 51 (33-77) years, with a male: 1.43: 1.00. There were 14 cases of stage Ⅲ (82.4%) in DS stage, 6 cases (35.3%) in stage B and 10 cases (58.8%) in stage Ⅲ of ISS. IgD light chain to λ common (16 cases), accounting for 94.1%. Blood, urine monoclonal light chain abnormalities increased, and serum total globulin and IgG, IgA, IgM levels were low or normal. At diagnosis, bone pain and bone destruction accounted for 41.2% and 47.1% respectively, anemia accounted for 70.6% and renal dysfunction accounted for 47.1%. 13 cases of bone marrow examination found abnormal plasma cell proliferation. Extramedullary infiltration (confirmed by pathology) accounted for 58.8%, of which 3 cases of thoracic or lumbar vertebral body adjacent to the presence of soft tissue mass, and 2 cases of paraplegia; 7 patients had pleural effusion; 1 patient after the sternum ; 1 case of thyroid and breast lesions. 6/13 cases of bortezomib-based chemotherapy, the survival curve showed a trend superior to the traditional chemotherapy, but the difference was not statistically significant (P = 0.212). Conclusion: The incidence of IgD type MM is low, but its invasiveness is high. IgD should be routinely detected by immunofixation to avoid misdiagnosis and misdiagnosis. Combined treatment with new drugs such as proteasome inhibitor may be beneficial to prolong the survival of patients.