Heyning F H, Hogendoorn P C W, Kramer M H H, Holland C T Q, Dreef E, Jansen P M
Department of Internal Medicine, Medical Center Haaglanden, The Hague, The Netherlands.
J Clin Pathol. 2009 Sep;62(9):820-4. doi: 10.1136/jcp.2008.063156.
To determine prognostic significance of immunohistochemical markers and investigate possible germinal centre (GC) derivation in primary lymphoma of bone (PLB).
Immunohistochemical expression of BCL-6, CD10, BCL-2, p53, CD30, CD44 and MUM-1 was studied in 36 patients with PLB. All cases were clinically staged and cases of secondary bone involvement of primary nodal lymphomas were excluded, prior to immunostaining. Clinical charts were reviewed for clinical symptoms and therapy given; survival post-biopsy was calculated.
All patients presented with pain and a palpable mass. The majority showed centroblastic-multilobated morphology; half of the cases (19/36) had a GC phenotype (CD10+BCL-6+ or CD10-BCL-6+MUM-1-), whereas 8/36 cases had a non-GC phenotype (CD10-BCL-6- or CD10-BCL-6+MUM-1+). Nine cases were of indeterminate phenotype (CD10-BCL-6+; MUM-1 not available). Eight of 22 evaluated patient samples showed immunoreactivity for MUM-1. Most patients (31/36) received combination therapy in the form of polychemotherapy and radiotherapy. The five-year overall survival was 75%. No significant difference in survival was found between the three different tumour phenotypes, or for the tested antigens individually. Age at presentation and stage of disease had a significant influence on five-year overall survival. Survival rates were 90% for the patients <60 years of age and 40% for those > or =60 years. Survival rates were 90% for stage I and 41% for stage IV.
This study illustrates the homogeneity of PLB. The majority of cases are of the GC phenotype which has a favourable prognosis.
确定免疫组化标志物的预后意义，并研究原发性骨淋巴瘤（PLB）中可能的生发中心（GC）来源。
对36例PLB患者进行BCL-6、CD10、BCL-2、p53、CD30、CD44和MUM-1的免疫组化表达研究。所有病例均进行了临床分期，在免疫染色前排除了原发性淋巴结淋巴瘤继发性骨受累的病例。回顾临床病历以了解临床症状和所给予的治疗；计算活检后的生存率。
所有患者均表现为疼痛和可触及肿块。大多数表现为中心母细胞-多叶形态；半数病例（19/36）具有GC表型（CD10+BCL-6+或CD10-BCL-6+MUM-1-），而8/36病例具有非GC表型（CD10-BCL-6-或CD10-BCL-6+MUM-1+）。9例为不确定表型（CD10-BCL-6+；MUM-1未检测）。22例评估患者样本中有8例显示MUM-1免疫反应性。大多数患者（31/36）接受了以多药化疗和放疗形式的联合治疗。五年总生存率为75%。三种不同肿瘤表型之间或单个检测抗原之间在生存率上未发现显著差异。发病年龄和疾病分期对五年总生存率有显著影响。年龄<60岁的患者生存率为90%，年龄≥60岁的患者生存率为40%。I期患者生存率为90%，IV期患者生存率为41%。
本研究说明了PLB的同质性。大多数病例为GC表型，预后良好。
