Chłoniaki agresywne z komórek B z podwójną/potrójną translokacją - double/triple hit lymphoma
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- 作者：Jan Walewski ; walewski@coi.pl" class="auth_mail" title="E-mail the corresponding author
- 关键词：double hit lymphoma ; double expressor lymphoma ; rearanżacje MYC ; BCL2 ; chłoniaki nieklasyfikowalne z komó ; rek B o cechach pośrednich między DLBCL i BL
- 刊名：Acta Haematologica Polonica
- 出版年：2015
- 出版时间：September 2015
- 年：2015
- 卷：46
- 期：4
- 页码：263-268
- 全文大小：336 K
文摘
Large B-cell lymphoma with cytogenetically detected rearrangement and/or increased copy number of MYC, BCL2, and/or BCL6 are frequently termed double/triple hit lymphoma (DHL/THL) due to molecular, biologic, and clinical similarities. In particular, patient outcome after standard therapy is disappointing; there is a high risk of central nervous system involvement, and resistance to second-line treatment in case of recurrent or progressive disease. DHL/THL are not recognized as a separate diagnostic entity, and should be diagnosed within the category of B-cell lymphoma unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (BCLU). In majority of cases, DHL/THL display molecular and phenotypic features of GCB (germinal center B-cell-like) cell of origin. There is also a distinct group of large B-cell lymphoma with overexpression of MYC (usually ≥40% cells positive), BCL2, and/or BCL6 (usually ≥50–70% cells positive), called double expressor (DE). These cases are in majority derived from non-GCB/ABC cell of origin. Similar to the DHL, prognosis in DE is poor, and second-line therapy in case of relapse or progression is unsuccessful. Limited, available clinical data suggest that a standard immunochemotherapy R-CHOP is unacceptable for both DHL and DE, and new approaches to therapy and new drugs are needed.