Zagouri Flora, Kastritis Efstathios, Symeonidis Argiris S, Giannakoulas Nikolaos, Katodritou Eirini, Delimpasi Sosana, Repousis Panagiotis, Terpos Evangelos, Dimopoulos Meletios A
Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Eur J Haematol. 2014 Apr;92(4):308-12. doi: 10.1111/ejh.12255. Epub 2014 Jan 26.
Immunoglobulin D (IgD) multiple myeloma is an uncommon variant of the disease probably associated with poorer prognosis. However, data on IgD myeloma patients treated in the novel agent era are lacking.
To assess the frequency and the specific characteristics and evaluate the outcome of patients with IgD myeloma, we analyzed the database of the Greek Myeloma Study Group.
Between January 2000 and December 2012, among the 1239 patients with symptomatic myeloma, 31 (2.5%) were diagnosed with IgD myeloma. The median age of patients with IgD myeloma was 65 yr (range 26-80 yr) versus 68 yr (range 23-96 yr) of all others, and 84% had lambda light chain (vs. 38% of the patients with other subtypes). Patients with IgD myeloma presented more often with features of high-risk disease, that is, with advanced ISS, high LDH, significant renal dysfunction, and large amounts of Bence Jones proteinuria. Response to primary therapy was similar to other patients, although there was a trend for better quality of responses in patients with IgD myeloma. The median survival of these patients was 51.5 months versus 50.7 months for patients of other subtypes. In a multivariate model to adjust for differences in prognostic features, IgD myeloma was not associated with a different prognosis.
The incidence of IgD myeloma is 2.5%. Although patients with IgD myeloma present more often with high-risk features, their outcome in the era of novel agents is similar to that of patients with other myeloma subtypes.
免疫球蛋白D（IgD）多发性骨髓瘤是一种罕见的疾病变体，可能与较差的预后相关。然而，关于新型药物时代接受治疗的IgD骨髓瘤患者的数据尚缺乏。
为评估IgD骨髓瘤患者的发病率、具体特征并评估其预后，我们分析了希腊骨髓瘤研究组的数据库。
结果。
2000年1月至2012年12月期间，在1239例有症状的骨髓瘤患者中，31例（2.5%）被诊断为IgD骨髓瘤。IgD骨髓瘤患者的中位年龄为65岁（范围26 - 80岁），而其他所有患者为68岁（范围23 - 96岁），并且84%的患者有λ轻链（其他亚型患者为38%）。IgD骨髓瘤患者更常表现出高危疾病特征，即国际分期系统（ISS）晚期、乳酸脱氢酶（LDH）高、明显肾功能不全和大量本周氏蛋白尿。对初始治疗的反应与其他患者相似，尽管IgD骨髓瘤患者的反应质量有更好的趋势。这些患者的中位生存期为51.5个月，其他亚型患者为50.7个月。在一个用于调整预后特征差异的多变量模型中，IgD骨髓瘤与不同的预后无关。
IgD骨髓瘤的发病率为2.5%。尽管IgD骨髓瘤患者更常表现出高危特征，但在新型药物时代，他们的预后与其他骨髓瘤亚型患者相似。
