17例IgD型多发性骨髓瘤临床分析

Clinical analysis on 17 cases of IgD multiple myeloma

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 characteristics and treatment options of IgD multiple myeloma（MM）and to improve the level of diagnosis and treatment.Method：Clinical characteristics,prognosis of stages and different treatments for survival of 17 patients diagnosed with IgD MM were retrospectively analyzed.Result：Of the 284 cases of newly diagnosed MM patients at the same period,17 cases of patients with IgD MM,accounting for 6.0%.The male-female ratio was 1.43∶1.00 with a median age of 51（33to 77）years.Fourteen patients（82.4%）were of stageⅢ by Durie Salmon while six patients（35.3%）of stage B.Ten patients（58.8%）were of stageⅢ by International Staging System.Ninety-four percent of patients had lambda light chains.Level of blood and urine monoclonal light chains was abnormally high and total serum globulin and IgG,IgA,IgM levels were low or normal.Bone pain and bone destruction at diagnosis accounted for 41.2% and 47.1%,respectively.Anemia and renal function impairment were present in 70.6% and 47.1% patients.Abnormal plasma cells were revealed in 13 patients by bone marrow examination.Extramedullary infiltration（confirmed by pathology）incidence of 58.8%,of which there were 3cases of thoracic or lumbar vertebrae adjacent soft tissue mass,and 2cases of paraplegia occurred;7cases with pleural effusion;1case of substernal mass and 1case of thyroid and breast placeholder.Six patients treated with chemotherapy based on bortezomib showed a better tendency of survival curves than that of traditional chemotherapy,but there were no statistically significant differences（P=0.212）.Conclusion：IgD MM is rare and has a higher invasion than other MM isotypes.Immunofixation electrophoresis should be routinely performed to detect IgD in order to avoid misdiagnosis.Treatment options including new drugs such as bortezomib may further improve the outcomes of these patients.