Hematology, Karolinska Institutet, Stockholm, Sweden.
Regional Cancer Centre West, Gothenberg, Sweden.
Eur J Haematol. 2017 Sep;99(3):216-222. doi: 10.1111/ejh.12907. Epub 2017 Jun 28.
Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not translate into better survival in EMP. In spite of treatment developments, the OS of primary PCL is still dismal (median of 11 months, 0% at 5 years). Hence, there is a great need for diagnostic and treatment guidelines as well as prospective studies addressing the role for alternative treatment options, such as allogeneic stem cell transplantation and monoclonal antibodies in the treatment of PCL.
孤立性浆细胞瘤（SP）和浆细胞白血病（PCL）是罕见的（3-6%）浆细胞疾病类型。进展为有症状多发性骨髓瘤（MM）的风险可能对 SP 的结局很重要。PCL 很罕见，预后不良。在这项研究中，我们使用旨在记录自 2008 年以来瑞典所有新诊断的浆细胞疾病的前瞻性观察性瑞典多发性骨髓瘤登记处，报告了 PCL/SP 的发病率和生存率，以及 SP 进展为 MM 的情况。孤立性骨浆细胞瘤（SBP）（n=124）和髓外浆细胞瘤（EMP）（n=67）的总生存率（OS）均优于 MM（n=3549）。SBP 进展为 MM 的比例高于 EMP（分别为 35%和 7%，2 年时），但这并未转化为 EMP 的生存获益。尽管治疗方法有所发展，但原发性 PCL 的 OS 仍然很差（中位 11 个月，5 年时为 0%）。因此，非常需要制定诊断和治疗指南，以及开展前瞻性研究，探讨异体干细胞移植和单克隆抗体等替代治疗方案在 PCL 治疗中的作用。
