摘要目的 探讨骨原发间变性大细胞淋巴瘤(ALCL)的临床病理学特征、诊断、鉴别诊断及预后.方法 回顾性分析12例确诊为骨原发性ALCL患者的临床病理资料,并进行免疫组织化学EnVision法染色和EB病毒原位杂交,对其临床病理学特征及预后情况进行分析.结果 12例患者男性7例,女性5例.发病年龄9～64岁,中位年龄17.5岁.就诊时患者均表现为骨痛,影像学检查为溶骨性骨破坏,包括8例单发和4例多发性骨破坏.发病部位依次为:脊柱7例、髂骨5例、骶骨2例,肱骨和锁骨各1例.组织学以普通型最为常见(8例),另有淋巴组织细胞型和小细胞型各2例.蛋白水平检测显示全部病例肿瘤细胞一致性强表达CD30,其中7例间变性淋巴瘤激酶(ALK)阳性,5例ALK阴性.10例获得随访,随访时间2～47个月,7例临床缓解,3例死亡.7例ALK阳性患者中5例获得随访,生存状况均良好;5例ALK阴性患者中3例死亡.结论 骨原发ALCL以儿童和青年男性多见,中轴骨为好发部位.ALK蛋白表达可能是提示骨原发ALCL预后良好的指标.

abstractsObjective To study the clinicopathologic features,differential diagnosis and prognosis of primary bone anaplastic large cell lymphoma(ALCL).Methods Twelve patients diagnosed with primary bone ALCL were retrospectively reviewed.The clinicopathologic features,immunohistochemic findings and results of in situ hybridization for EB virus were analyzed.Results Of the 12 patients,the male-to-female was 7∶5 with a median age of 17.5 years (range from 9 to 64 years).Bone pain was the presenting symptom in all patients.Radiographic examination demonstrated solitary osteolytic lesion in 8 patients and multiple lesions in the rest 4 patients.Spine (7 cases) was the most common site to be involved,followed by ilium (5 cases),sacrum (2 cases),humerus (1 case) and collarbone (1 case).Ten patients were available with the follow-up data including 5 ALK-positive and 5 ALK-negative patients,and the follow-up time was 2 to 47 months.Interestingly,the 3 dead patients were ALK-negative whereas 5 of 7 ALK-positive patients achieved remission.Conclusions Primary bone ALCL is a rare type of non-Hodgkin lymphoma and it more frequently involves the axial skeleton.Boys and young males are more commonly affected.Patients usually present at an early stage and have a relatively favorable prognosis.Expression of ALK protein may be associated with a favorable prognosis in primary bone ALCL.