A Case of Double Expresser Diffuse Large B Cell Lymphoma Treated with R-CODOX-M/R-IVAC

Abstract

Diffuse Large B Cell Lymphoma (DLBCL) is a heterogeneous disease with a variety of chromosomal abnormalities contributing to differences in management. While it is known that Double Hit Lymphomas (DHL) warrant more aggressive chemotherapy regimens, debate remains on how to treat Double Expresser Lymphomas (DEL). We present a case of a DEL treated with an aggressive regimen of 2 alternating cycles of R-CODOX-M (rituximab, cyclophosphamide, doxorubicin, vincristine and methotrexate) and R-IVAC (rituximab, ifosfamide, etoposide and high dose cytarabine). The regimen resulted in a significant response to treatment with marked reduction in tumor size and avidity, and an acceptable side effect profile. There was, however, residual metabolic activity on repeat PET CT scan. After consolidation with 36 Grey radiotherapy, a PET CT demonstrated a complete metabolic response. Debate remains regarding treatment approaches in DEL. Our case supports the categorization of DEL alongside DHL as resistant lymphomas requiring a more aggressive regimen than standard therapy.

High-Grade B-Cell Lymphoma Not Otherwise Specified (HGBL, NOS) in the Maxillary Sinus Mimicking Periapical Inflammation: Case Report and Review of the Literature

“…Therefore, the WHO 2008 statement defined these groups as B-cell lymphomas, unclassifiable, with features intermediate between DLBCL and BL, but this definition is no longer recommended [13]. HGBL is a special class of aggressive mature lymphomas, and it includes two subtypes: (1) double or triple hit HGBL lymphomas, and (2) high-grade B-cell lymphoma not otherwise specified (HGBL, NOS), or double-expressor lymphomas (DEL) [11,[14][15][16]. If MYC and BCL-2 and/or BCL-6 rearrangements are present, they are categorized as HGBL with MYC and BCL-2 and/or BCL-6 rearrangements.…”

“…The protein encoded by BCL-2 inhibits apoptosis. The expression of BCL-6, a transcriptional repressor, helps in the development of lymphoma [15,18]. Both subtypes, especially the HGBL, NOS, are rarely diagnosed [12,19], and while other mature B-cell lymphomas such as DLBCL have an average of 70% complete remission and a high average 5-year survival rate of 60% [1,20], the prognosis of HGBL, NOS is poor [14,[21][22][23].…”