19例原发骨淋巴瘤临床病理特点及预后分析

Clinical and pathological analysis of 19 patients with primary bone lymphoma

目的：分析原发骨淋巴瘤（PBL）的临床病理特征及预后相关因素。方法：回顾性分析19例PBL患者的临床病理资料,分析总结其临床病理特征及预后因素。结果：119例患者中位发病年龄为51（15~78）岁。主要病理类型为弥漫大B细胞淋巴瘤,共15例（78.9%）,其中非生发中心型9例（60.0%）,生发中心型6例（40.0%）。首发症状最常见为骨痛或活动障碍（18/19,94.7%）。单病灶骨受累14例（73.7%）,多病灶骨受累5例（26.3%）。单病灶受累最常见部位是股骨,有4例（28.6%）,其次为骨盆3例（21.4%）、脊椎3例（21.4%）。Ann Arbor临床分期Ⅰ~Ⅱ期占52.6%,Ⅳ期占47.4%。15例行PET-CT检查,其中Ⅳ期7例（46.7%）。217例接受联合化疗,另2例仅接受手术剔除修复治疗。近期疗效显示9例（52.9%）达完全缓解（CR）,7例（41.2%）达部分缓解,1例（5.9%）疾病进展。3年龄与CR率有关（P〈0.05）,而性别、临床分期、IPI评分、肿瘤细胞起源、血清β2-微球蛋白、乳酸脱氢酶、铁蛋白水平及是否应用利妥昔单抗体与CR率无明显相关性（P〉0.05）。中位随访15（2~60）个月,其中16例存活,2例死亡,1例失访;中位无进展时间为16（4~60）个月。年龄及应用利妥昔单抗是重要的预后因素,初始诱导化疗CR患者可能总生存更佳,但差异无统计学意义（P=0.093）;肿瘤细胞起源与总生存无关。结论：PBL主要病理类型为弥漫大B细胞淋巴瘤,年龄与治疗CR率相关,年龄及联合利妥昔单抗治疗是重要的预后相关因素。

Objective：To analyze the clinical and pathological features,prognostic factors and treatment outcomes of primary bone lymphoma（PBL）.Method：Clinical data of 19 patients with PBL were analyzed retrospectively.Result：1The median age was 51（15to 78）among 19 evaluable patients.The majority of cases were presented with diffuse large B cell lymphoma（DLBCL）and contained 9non-GCB patients and 6GCB patients.Fourteen patients were unifocal bone disease involvement and the other 5were multifocal.Femur was found to be the most commonly affected site in unifocal PBL patients（28.6%）at the time of presentation,and then the pelvis（21.4%）and spine（21.4%）.According to Ann Arbor classification system,52.6% patients were classified in stageⅠtoⅡ and 47.4% patients stage Ⅳ.Among 15 patients who had underwent PET-CT examination,7cases were confirmed as stageⅣ.2Seventeen of 19 cases were treated with chemotherapy and the result showed 9cases（52.9%）in complete remission（CR）,7cases（41.2%）in partial remission and 1case（5.9%）in disease progression.The other 2cases just underwent surgery for repairing treatment.3The correlation of age（P〈0.05）,rather than sex,stage,IPI score,the origin of the tumor cells,LDH,β2-MG,serum ferritin levels and the application of rituximab,and the CR rate was statically significant.Sixteen cases survived,2cases died and 1case was lost to follow up after a median follow-up of 15（2to 60）months.The median progression-free time was 16（4to 60）months.Age and the application of rituximab were important prognostic factors.The patients who got CR during initial induction chemotherapy may have a better overall survival,but there was no statistical difference（P=0.093）.There was no relationship between overall survival and the origin of tumor cells.Conclusion：The most common histologic type of PBL is DLBCL.Age is related to CR rate.Age and application of rituximab are independent prognostic factors for overall survive.