骨外（髓外）浆细胞瘤：32 例中国病例的临床病理和免疫表型研究。

BACKGROUND

Extraosseous plasmacytoma, so called extramedullary plasmacytoma (EMP) is relatively rare in China. The aim was investigate the clinicopathologic features of EMP and the role of Immunophenotype and genotype detection in diagnosis of EMP.

METHODS

Thirty-two cases of EMP were investigated retrospectively by histopathology, immunophenotype, genotype and survival analysis.

RESULTS

Clinically, the mean age of the patients was 53.4. Most of the patients received no treatment after the diagnosis was established, and the prognosis was relatively poor. Histologically, in 40% of the cases, the neoplastic cells were grade II or III. The neoplastic cells expressed one or more PC associated antigens. The immunophenotype of EMP and inflammation of sinonasal regions with numerous PC infiltrations were compared and showed some difference in expression of CD45, CD27, CD44v6 and Bcl-2 as well. Ig light chain restriction was detected in 87.5% of the cases.

CONCLUSIONS

we described 32 Chinese cases of EMP, compare with that reported in the literature, some differences are presented, including higher percentage of grade II and III cases, clinically inconsistent treatment and management as well as poor outcome of the disease.

背景

骨外浆细胞瘤，又称为髓外浆细胞瘤（EMP），在中国较为罕见。本研究旨在探讨 EMP 的临床病理特征，以及免疫表型和基因型检测在 EMP 诊断中的作用。

方法

通过组织病理学、免疫表型、基因型和生存分析回顾性研究了 32 例 EMP 病例。

结果

临床方面，患者的平均年龄为 53.4 岁。大多数患者在确诊后未接受治疗，预后相对较差。组织学上，40%的病例中肿瘤细胞为 II 级或 III 级。肿瘤细胞表达一种或多种与 PC 相关的抗原。EMP 的免疫表型与鼻旁窦区域的炎症伴大量 PC 浸润进行比较，在 CD45、CD27、CD44v6 和 Bcl-2 的表达上也存在一些差异。87.5%的病例检测到免疫球蛋白轻链受限。