Ramadan K M, Shenkier T, Sehn L H, Gascoyne R D, Connors J M
Division of Hematology.
Division of Medical Oncology.
Ann Oncol. 2007 Jan;18(1):129-135. doi: 10.1093/annonc/mdl329. Epub 2006 Oct 3.
Primary bone lymphoma (PBL) is a distinct clinicopathological entity. Although PBL has been reviewed in several small studies, few reflect recent improvements in primary treatment.
We used the British Columbia Cancer Agency Lymphoid Cancer Database to identify all patients with PBL (1983-2005). All were staged in a uniform manner and treated with era-specific protocols.
We identified 131 patients with a median age of 63 years (18-87). One third had disease in long bones and another one third had disease in the spine, of which half presented with spinal cord compression. Patients with diffuse large-cell lymphoma (DLCL) (n=103, 79%) had 5- and 10-year overall survivals (OS) of 62% and 41%, respectively. Multivariate analysis identified three prognostic groups: age<60 with International Prognostic Index (IPI) 1-3 (n=43), age>or=60 with IPI 0-3 (n=23) and age>or=60 with IPI 4-5 (n=33), with markedly different 5-year OS of 90%, 61% and 25%, respectively (P<0.0001). Neither primary site nor pathological fracture at presentation had an impact on OS. The 3-year progression-free survival in patients who received rituximab plus combination chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOPR) chemotherapy was 88% compared with 52% in those who received CHOP-like chemotherapy without rituximab (P=0.005). The 10-year OS for those with advanced-stage disease who received irradiation plus chemotherapy was 25% versus 56% for those who received chemotherapy alone (P=0.025). Patients received irradiation if spinal cord compression was present or residual disease at the end of chemotherapy was thought to require it.
PBL is usually of DLCL type and has an improved outcome with CHOPR. Younger patients with good IPI score have a favorable prognosis.
原发性骨淋巴瘤（PBL）是一种独特的临床病理实体。尽管已有几项小型研究对PBL进行了综述，但很少有研究反映出原发性治疗的近期进展。
我们使用不列颠哥伦比亚癌症机构淋巴瘤数据库来识别所有PBL患者（1983 - 2005年）。所有患者均以统一方式分期，并采用特定时代的方案进行治疗。
我们识别出131例患者，中位年龄为63岁（18 - 87岁）。三分之一的患者病变位于长骨，另外三分之一位于脊柱，其中一半伴有脊髓压迫。弥漫性大细胞淋巴瘤（DLCL）患者（n = 103，79%）的5年和10年总生存率（OS）分别为62%和41%。多变量分析确定了三个预后组：国际预后指数（IPI）为1 - 3且年龄<60岁的患者（n = 43）、IPI为0 - 3且年龄≥60岁的患者（n = 23）以及IPI为4 - 5且年龄≥60岁的患者（n = 33），其5年OS分别为90%、61%和25%，差异显著（P<0.0001）。初诊时的原发部位和病理性骨折均对OS无影响。接受利妥昔单抗联合环磷酰胺、阿霉素、长春新碱和泼尼松（CHOPR）化疗的患者3年无进展生存率为88%，而接受不含利妥昔单抗的CHOP样化疗的患者为52%（P = 0.005）。接受放疗加化疗的晚期疾病患者10年OS为25%，而单纯接受化疗的患者为56%（P = 0.025）。如果存在脊髓压迫或化疗结束时的残留疾病被认为需要放疗，则患者接受放疗。
PBL通常为DLCL型，采用CHOPR治疗预后有所改善。IPI评分良好的年轻患者预后良好。
