Rearrangements of bcl-6, bcl-2, c-myc and 6q deletion in B-diffuse large-cell lymphoma: clinical relevance in 71 patients.
Rearrangements of bcl-6, bcl-2, c-myc and 6q deletion in B-diffuse large-cell lymphoma: clinical relevance in 71 patients.
复制标题
B 弥漫性大细胞淋巴瘤中 bcl-6、bcl-2、c-myc 和 6q 缺失的重排：71 名患者的临床相关性。
DOI：
10.1023/a:1008201729596
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
L. Resegotti
中科院分区：
文献类型：
--
作者：
U. Vitolo;G. Gaidano;B. Botto;G. Volpe;E. Audisio;M. Bertini;R. Calvi;R. Freilone;D. Novero;L. Orsucci;C. Pastore;D. Capello;G. Parvis;C. Sacco;V. Zagonel;A. Carbone;Umberto Mazza;G. Palestro;G. Saglio;L. Resegotti
背景
B 弥漫性大细胞淋巴瘤 (DLCL) 与一些分子病变相关，但此类病变作为预后标志物的作用仍存在争议。本报告涉及 B-DLCL 中 bcl-6、bcl-2、c-myc 重排和 6(q) 缺失的频率和临床相关性的调查。
患者和方法
对 71 名 B-DLCL 患者诊断时收集的淋巴结或骨髓样本进行了分析，分析了这些遗传病变的存在，所有患者均接受含蒽环类药物的化疗方案治疗。
结果
11 名患者 (15%) 发现 bcl-6 重排，12 名患者 (17%) 发现 bcl-2 重排，10 名患者 (14%) 发现 6(q) 缺失，4 名患者 (6%) 发现 c-myc 重排。具有重排bcl-6的患者往往比具有种系bcl-6的患者患有更具侵袭性的疾病（根据IPI标准，中高/高风险：73%对43%），但两组之间的三年生存率没有差异（62%对42%）。重排患者和种系 bcl-6 患者的受累结外部位数量相似。 bcl-2 重排患者的疾病侵袭性似乎比种系 bcl-2 患者低（低/中低风险 75% vs. 47%），三年生存率略高（70% vs. 41%），但差异并不显着。有或没有 6(q) 缺失的两组都有相似的临床特征和结果。四名发生 c-myc 重排的患者患有侵袭性疾病，并且情况不佳。
结论
B-DLCL 的分子病变分析可能有助于更好的诊断定义；然而，在这项研究中，我们无法证明所评估的遗传病变对临床结果有显着影响。
BACKGROUND
B-diffuse large-cell lymphomas (DLCL) have been associated with some molecular lesions, but the role of such lesions as prognostic markers is still controversial. This report concerns an investigation of the frequency and clinical correlation of bcl-6, bcl-2, c-myc rearrangements and 6(q) deletions in B-DLCL.
PATIENTS AND METHODS
The presence of these genetic lesions was analyzed in samples of lymph nodes or bone marrow collected at diagnosis in 71 patients with B-DLCL, all treated with an anthracycline-containing chemotherapy regimen.
RESULTS
Rearrangement of bcl-6 was found in 11 patients (15%), rearranged bcl-2 in 12 (17%), 6(q) deletions in 10 patients (14%) and c-myc rearrangement in four (6%). Patients with rearranged bcl-6 tended to have a more aggressive disease than patients with germ-line bcl-6 (intermediate-high/high risk according to IPI criteria: 73% vs. 43%), but there were no differences in three-year survival rates (62% vs. 42%) between the two groups. The numbers of involved extranodal sites were similar in patients with rearranged and those with germ-line bcl-6. Patients with bcl-2 rearrangement appeared to have a less aggressive disease than those with germ-line bcl-2 (low/ low-intermediate risk 75% vs. 47%) and a slightly better three-year survival rate (70% vs. 41%) but again the difference was not significant. Both groups with or without 6(q) deletion had similar clinical characteristics and outcomes. The four patients with c-myc rearrangement had aggressive disease and did poorly.
CONCLUSIONS
The analysis of molecular lesions in B-DLCL may be useful for a better diagnostic definition; however, in this study we were unable to show that the evaluated genetic lesions had a significant impact on clinical outcome.
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