原发性骨非霍奇金淋巴瘤临床病理预后和病因学分析

目的探讨原发性骨非霍奇金淋巴瘤（PNHLB）的临床病理特征、预后指标及病因学。方法复习17例PNHLB患者的临床资料,同时进行免疫组织化学EnVision法检测免疫标志物、原位杂交检测EBER及PCR检测bcl-2／JH基因重排,并对血清LDH、治疗、国际预后指数（IPI）、免疫标志物与预后的关系进行分析。结果17例PNHLB以弥漫性大B细胞淋巴瘤为主（94．1％）,患者的5年生存率为68．8％,IPI高危类、bcl-2过表达对预后不利（2者的P值分别为0．031和0．028）,治疗方式和CD10、MUM-1、bcl-6的表达对预后的判断差异无统计学意义（P〉0．05）。8例人类B-珠蛋白基因扩增阳性的骨DLBCL患者中1例Bcl-2／JH基因重排扩增阳性。EBER原位杂交仅1例阳性。结论PNHLB预后较好,IPI及免疫组织化学检测bcl-2过表达是判断预后的指标。EB病毒与病因无相关性。

Clinicopathologic features, prognosis and etiology of primary non-Hodgkin lymphoma of bone

OBJECTIVE: To study the clinicopathologic features, prognostic indicators and possible etiology of primary non-Hodgkin lymphoma of bone (PNHLB). METHODS: The clinicopathologic features of 17 cases of PNHLB were reviewed. In-situ hybridization for Epstein-Barr virus early RNA (EBER) and polymerase chain reaction for bcl-2/JH gene rearrangement were performed using paraffin-embedded materials. The correlation between serum lactic dehydrogenase level, treatment options, international prognostic indicator (IPI) and immunophenotype with clinical outcome were analyzed. RESULTS: The majority of the 17 cases studied was diffuse large B-cell lymphoma (94.1%). The 5-year survival rate was 68.8%. Unfavorable prognostic factors included high-risk IPI (P = 0.031) and bcl-2 overexpression (P = 0.028). Treatment options and expression of CD10, MUM-1 or bcl-6 did not correlate with clinical outcome (P > 0.05). Only 1 patient was positive for EBER, as demonstrated by in-situ hybridization. CONCLUSIONS: The clinical outcome of PNHLB is relatively favorable. IPI and bcl-2 expression may serve as useful prognostic indicators. EBV is likely not related to pathogenesis of this type of lymphoma.