CHARACTERISTICS AND TREATMENT OUTCOMES OF AGGRESSIVE HIGH GRADE B CELL LYMPHOMA WITH MYC AND BCL 6 REARRANGEMENTS

According to the International Consensus Classification of Lymphomas (Blood 2022: 140: 1229–53), a new provisional entity high grade B cell lymphoma with MYC and BCL 6 rearrangements (HGBCL-DH-BCL6) was introduced. HGBCL-DH-BCL6 occurred very rare, in less than 10% patients HGBCL rearrangements. Here we assessed clinicopathological features treatment outcomes HGBCL-DH-BCL6. We retrospectively collected clinic-pathological data MYC/BLC6-rearrangements. All cases were evaluated by histopathological/immunohistochemical/flow-cytometry examination (IHC/FCM), broad panel antibodies, including CD10/CD38/ MYC/LMO2/ BCL2/BCL6. Gene status (MYC/BCL2/BCL6) examined using fluorescence situ hybridization (FISH), karyotype successfully achieved 70% patients. identified 18 MYC-rearrangement (median MYC-R: 49% cells; range: 5%–93%), BCL6-rearrangements BCL6-R: 50% 5%–93%) no BCL2- found. Expression MYC-protein found only 22% Eleven seven patients, respectively, had DLBCL BCL-u morphology. Median age at diagnosis 57 years (range: 18–84). Advanced stage, extranodal disease, increased dehydrogenase lactate 78%, 33%, 83% respectively. Only 15 received immunochemotherapy: 8 treated R-CHOP CNS prophylaxis, other 7 more intensive therapy: GMALL, CODOX/IVAC or DAEPOCH-R. Six consolidative auto-SCT. Morphology intensity immunochemotherapy impact on response survival. 36% complete remission. overall survival (OS) progression free (PFS) 5.6 months 3.0 months, OS PFS for 1 year 34% 28% Actually, 4 are alive active disease. is rare aggressive B-cell lymphoma, often without IHC-protein deletion, advanced disease presenting diagnosis. The poor 40% patient from one Intensive therapy has not improved results. Keywords: non-Hodgkin pathology classification lymphomas No conflicts interests pertinent abstract.