CAJ | 학술논문

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.
목적 탐토골원발간변성대세포림파류(ALCL)적림상병이학특정、진단、감별진단급예후.방법 회고성분석12례학진위골원발성ALCL환자적림상병리자료,병진행면역조직화학EnVision법염색화EB병독원위잡교,대기림상병이학특정급예후정황진행분석.결과 12례환자남성7례,녀성5례.발병년령9～64세,중위년령17.5세.취진시환자균표현위골통,영상학검사위용골성골파배,포괄8례단발화4례다발성골파배.발병부위의차위:척주7례、가골5례、저골2례,굉골화쇄골각1례.조직학이보통형최위상견(8례),령유림파조직세포형화소세포형각2례.단백수평검측현시전부병례종류세포일치성강표체CD30,기중7례간변성림파류격매(ALK)양성,5례ALK음성.10례획득수방,수방시간2～47개월,7례림상완해,3례사망.7례ALK양성환자중5례획득수방,생존상황균량호;5례ALK음성환자중3례사망.결론 골원발ALCL이인동화청년남성다견,중축골위호발부위.ALK단백표체가능시제시골원발ALCL예후량호적지표.
Objective 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.