IgD型多发性骨髓瘤18例临床特征和生存分析

目的探讨IgD型多发性骨髓瘤(MM)的临床表现与实验室检查特点、对治疗的反应、生存及预后情况。方法回顾性分析中山大学附属第一医院1996-01-01—2010-05-30收治的18例IgD型MM患者的临床资料。结果 IgD型MM占所有MM的4.4%,起病中位年龄54岁(36～70岁)。18例IgD型MM中,IgD-λ型16例(88.9%),IgD-κ型2例(11.1%);ISS分期中,Ⅲ期10例(占55.6%);DS分期中,18例患者均为Ⅲ期;2例(11.1%)合并淀粉样变,4例(22.2%)伴有髓外浸润;2例(11.1%)起病时有高钙血症;10例(55.6%)有肾功能损害。10例接受≥4个疗程方案化疗,总有效率60.0%;达平台中位时间为5.2个月,中位疾病进展时间为7.8个月,中位生存时间为13.0个月。结论 IgD型MM是一种侵袭性疾病,诊断时多处于疾病晚期,临床表现和实验室检查与其他亚型MM有较大差异,对治疗反应差,生存期短,预后差。

Immunoglobulim D multiple myeloma:analysis of clinical features and survival time

Objective To investigate the clinical features,laboratory findings,treatment response,survival and patient outcomes with immunoglobulin D multiple myeloma(IgD MM). Methods The clinical data from 18 cases of IgD MM registered to First Affiliated Hospital of Sun Yat-sen University were retrospective analyzed. Results IgD MM accounted for 4.4% of all MM patients in our hospital.The median age at onset of IgD MM was 54 years old(range:36～70 years).Of the 18 patients,IgD-λ MM accounted for 88.9% and IgD-κ MM for 11.1%.Stage III was noted in 10 patients(55.6%) by International Staging System(ISS),and in all(100%) patients by Durie-Salmon staging system.Two patients(11.1%) were with amyloidosis,4 with extramedullary infiltration,2(11.1%) with hypercalcemia at onset of the disease,and 10(55.6%) with renal dysfunction.A total of 10 patients received at least four cycles of chemotherapy with a response rate being 60.0%.The median time to plateau phase was 5.2 months,median time to progression 7.8 months,and the median overall survival time was 13.0 months. Conclusion IgD MM is an aggressive disease that is usually detected at an advanced stage.Clinic features and laboratory findings of IgD MM differ greatly from other types of MM.Patients with IgD MM usually respond unfavorably to treatment,showing a short survival time and poor prognosis.