伴有MYC、bcl-2和/或bcl-6重排的高级别B细胞淋巴瘤临床病理学特征

目的:探讨伴有MYC、bcl-2和/或bcl-6重排的高级别B细胞淋巴瘤（HGBL）的临床病理学特征。方法:收集2017年1月至2020年9月上海交通大学医学院附属瑞金医院病理科经MYC、bcl-2和bcl-6基因荧光原位杂交（FISH）检测的大B细胞淋巴瘤1 138例。对诊断为伴有MYC、bcl-2和/或bcl-6重...

Clinicopathological features and prognosis of high-grade B-cell lymphoma with MYC and bcl-2 and/or bcl-6 rearrangements

Objective To investigate the clinicopathological characteristics and prognosis of high-grade B-cell lymphoma(HGBL)involving combined rearrangements of MYC,bcl-2 and bcl-6.Methods A total of 1138 cases of large B cell lymphoma(LBL)that were treated at the Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine from January 2017 to September 2020 were analyzed using fluorescence in situ hybridization(FISH)with probes against MYC,bcl-2 and bcl-6.The clinical and pathological data of the 45 patients with HGBL that had rearrangements of MYC and bcl-2 and/or bcl-6 were collected and retrospectively analyzed.Results Among the 1138 LBL,45(4.0%)cases had combined rearrangements of MYC,bcl-2 and/or bcl-6 that included 6 HGBL cases with MYC,bcl-2 and bcl-6 rearrangements,14 HGBL cases with MYC and bcl-2 rearrangements,and 25 HGBL cases with MYC and bcl-6 rearrangements.Of these 45 patients,29 patients were male,and 16 patients were female,aged 29 to 83 years.HGBL with MYC,bcl-2 and bcl-6 rearrangements and HGBL with MYC and bcl-2 rearrangement were reclassified as the germinal center B-cell(GCB)subtype using the Hans algorithm.HGBL with MYC and bcl-6 rearrangement were reclassified as the GCB subtype(68.0%)and the non-GCB subtype(32.0%).The vast majority of HGBL cases had a high Ki-67 proliferation index.Most HGBL patients had advanced stage disease with a high IPI score and an increased LDH level.Also,some patients had clinical features including elevated plasmaβ2-microglobulin levels,B symptoms,and bone marrow involvement.The IPI scores and LDH levels were significantly different between the HGBL cases with MYC,bcl-2 and bcl-6 rearrangements and the HGBL cases with MYC and bcl-6 rearrangements(P<0.05).Compared with the HGBL cases with MYC,bcl-2 and bcl-6 rearrangements,the HGBL cases with MYC and bcl-2 or bcl-6 rearrangements had a lower incidence of bone marrow involvement(P<0.05).There were no significant differences in the prognosis among HGBL cases with MYC,bcl-2 and bcl-6 rearrangements,the cases with MYC and bcl-2 rearrangements,and the cases with MYC and bcl-6 rearrangements(P>0.05).Conclusions HGBL with MYC,bcl-2 and/or bcl-6 rearrangements are rare types of B-cell lymphoma with high degree of malignancy and have a short overall survival.To reduce misdiagnosis and improve diagnostic accuracy,it is necessary to assess the patients′clinical features and conduct histopathological,immunohistochemical and FISH analyses.