摘要目的 探讨骨孤立性浆细胞瘤(SPB)的临床病理特征,了解免疫表型在SPB的病理诊断和鉴别诊断中的意义和作用.方法 收集1990-2008年21例SPB的临床病理资料并进行回顾性分析,应用免疫组织化学(EnVision或EliVision法)检测17种抗原的表达情况,并用半套式PCR技术,以免疫球蛋白重链通用型引物,以及BIOMED-2系统引物IgK和IgL进行免疫球蛋白基因重排检测.结果 21例SPB患者年龄36～72岁,中位年龄50岁.主要病变部位为中轴骨骼(14例,66.7%),其次为四肢骨骼(7例,33.3%).5例有血清Ig升高,其中3例为IgA型,2例为IgG型.临床表现与肿瘤部位有关,有局部疼痛、脊神经压迫征和病理性骨折等.所有病例均表现为局部孤立性占位病变伴骨质破坏.组织学分级:21例中Ⅰ、Ⅱ和Ⅲ级者分别为12例(57.1%)、5例(23.8%)和4例(19.0%).免疫表型:所有病例之瘤细胞均表达两种及以上浆细胞抗原,如CD138、CD38和浆细胞抗体;均不表达CD19和CD20,CD79a表达率为23.8%(5/21).CD56、CD27和CD44v6的表达率分别为57.1%(12/21)、15.0%(3/20)和23.8%(5/21).21例SPB中12例(57.1%)检出IgH基因克隆性重排.12例(57.1%)有随访,7例死亡,5例存活;其中3例发展为多发性骨髓瘤并已死亡.结论 SPB以骨的孤立性占位伴疼痛为其临床特征,诊断需排除多发性骨髓瘤髓外浸润之可能.免疫表型及Ig基因重排检测在该肿瘤的诊断中起重要作用.

abstractsObjective To investigate clinicopathologic features of solitary plasmacytoma of bone(SPB) and the role of immuno-phenotype and immunoglobulin gene rearrangement detection in the diagnosis and differential diagnosis of SPB.Methods A total of 21 cases of SPB were selected during a period from 1990 to 2008.A retrospective clinicopathologic study and immunohistochemistry (EnVision or EliVision methods) of 17 antigens were performed.In addition, universal IgH (FR3A/LJH/VLJH) primers and BIOMED-2 PCR multiplex tubes were used for IgK and IgL rearrangement analysis.Results The age of patients ranged from 36 to 72 years with a media of 50 years.Axial skeleton was the most common site of involvement, accounting for 66.7% of the cases ( 14 of 21 ), followed by the extremities of 33.3%(7 cases).Low serum level of M-components was found in 5 cases, including two of IgG type (21.4 g/L)and three of IgA type.Clinical manifestations were closely related to the anatomic sites involved, such as pain due to bone destruction, symptoms and signs caused by compression of spinal cord or nerve root, and pathological fracture.All cases presented as a solitary osteolytic lesion.According to the histological grading criteria, grade Ⅰ tumor was seen in 12 of 21 cases (57.1%).The remaining were grade Ⅱ (5 cases,23.8% ) and grade Ⅲ (4 cases, 19.0% ).Immunohistochemically, the neoplastic cells expressed two or more plasma cell antigens, including CD138, CD38 and PC, but no CD19 and CD20.CD79a expression detected in 23.8% (5/21) of the cases.Expression of CD56, CD27 and CD44v6 were 57.1% ( 12/21 ),15.0% (3/20) and 23.8% (5/21), respectively.Follow-up data were available in 12 of the 21 patients (57.1% ).Five patients were alive and 7 died.Three patients developed multiple myeloma (MM) and died of the tumor.Conclusions SPB is a rare tumor with bone pain as the most common presenting symptom due to bone destruction.The diagnosis of EMP can only be established after exclusion of an extramedullay invasion by MM.Immuuophenotype and IgH gene rearrangement analysis play important roles in the diagnosis of SPB.