Haematology Department, Belfast City Hospital, Lisburn Road, Belfast BT9 7AD, UK.
Haematologica. 2010 Dec;95(12):2126-33. doi: 10.3324/haematol.2010.022848. Epub 2010 Oct 22.
As rare myelomas, i.e. the IgD, IgE, IgM and non-secretory forms, constitute only a small proportion of any study, relatively little is known about their prognosis in the era of peripheral stem cell transplantation.
We used the European Group for Blood and Marrow Transplantation Myeloma Database to compare the outcome following autologous transplantation of over 20,000 patients with common myelomas (IgG, IgA and light chain myeloma) with the outcome of patients with rare myelomas: 379 IgD, 13 IgE, 72 IgM and 976 non-secretory cases.
The study confirms the multiple adverse prognostic factors seen in IgD myeloma. Somewhat surprisingly, patients with IgD and non-secretory myeloma both had higher complete remission rates before and after transplantation than patients with common myelomas. However, while the overall survival of patients with non-secretory myeloma was similar to that of the patients with common myelomas, the survival of patients with IgD myeloma was significantly worse (although better than survival rates reported for non-transplanted patients); this was due to higher transplant-related mortality and relapse/progression rates. The post-transplantation survival of patients with IgE or IgM myeloma appears to be very poor.
This study provides data on the biological features of rare myelomas. The overall survival of patients with IgD, IgE or IgM myeloma is poor following autologous transplantation but substantially better than that reported for patients who were not transplanted.
由于罕见骨髓瘤,如 IgD、IgE、IgM 和非分泌型,仅占任何研究的一小部分,因此对于在外周干细胞移植时代它们的预后相对知之甚少。
我们使用欧洲血液和骨髓移植骨髓瘤数据库,将超过 20000 例常见骨髓瘤(IgG、IgA 和轻链骨髓瘤)患者接受自体移植后的结果与罕见骨髓瘤患者的结果进行比较:379 例 IgD、13 例 IgE、72 例 IgM 和 976 例非分泌型病例。
该研究证实了 IgD 骨髓瘤中存在多种不良预后因素。有些令人惊讶的是,IgD 和非分泌型骨髓瘤患者在移植前后的完全缓解率均高于常见骨髓瘤患者。然而,虽然非分泌型骨髓瘤患者的总生存率与常见骨髓瘤患者相似,但 IgD 骨髓瘤患者的生存率明显较差(尽管优于未移植患者的报告生存率);这是由于移植相关死亡率和复发/进展率较高。IgE 或 IgM 骨髓瘤患者的移植后生存率似乎非常差。
本研究提供了罕见骨髓瘤生物学特征的数据。IgD、IgE 或 IgM 骨髓瘤患者在接受自体移植后的总生存率较差,但明显优于未接受移植患者的报告生存率。