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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。
Objective To investigate the clinical features, laboratory features, response to therapy, survival and prognosis of IgD multiple myeloma (MM). Methods The clinical data of 18 cases of IgD MM patients admitted to the First Affiliated Hospital of Sun Yat-sen University from January 1996 to January 2010 were retrospectively analyzed. Results IgD MM accounted for 4.4% of all MM, the median onset age of 54 years (36 to 70 years old). In 18 cases of IgD type MM, 16 cases (88.9%) of IgD-λ and 2 cases (11.1%) of IgD-κ were in ISS stage, 10 cases (55.6%) of stage Ⅲ in ISS stage, 18 cases 2 cases (11.1%) had amyloidosis, 4 cases (22.2%) had extramedullary infiltration, 2 cases (11.1%) had hypercalcemia on onset, and 10 cases (55.6%) had Kidney damage. Ten patients received ≥4 cycles of chemotherapy, with a total effective rate of 60.0%. The median time to reach the platform was 5.2 months. The median progression time was 7.8 months and the median survival time was 13.0 months. Conclusion IgD type MM is an aggressive disease. It is often diagnosed with advanced disease. Clinical manifestations and laboratory tests are quite different from those of other subtypes. It has poor response to treatment, short survival period and poor prognosis.