title: [Successful treatment with dose-adjusted EPOCH-R for triple-hit lymphoma having BCL2, BCL6 and MYC translocations].
abstract: Double- and triple-hit lymphomas (DHL/THL), high-grade B-cell lymphomas with an extremely poor prognosis, are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint. The successful use of dose-adjusted (DA) EPOCH-R in patients with MYC-positive lymphoma and Burkitt lymphoma (BL) was recently reported. A 74-year-old man with acute renal dysfunction and hyperkalemia was transferred to our emergency center by ambulance. PET-CT revealed a left renal hilar mass enveloping the abdominal para-aortic domain and bladder and hydronephrosis. High (18)F-FDG uptake revealed lymph node, peritoneum, and multiple bone metastases. Analysis of the bone marrow aspirate revealed abnormal lymphoid cells with deeply basophilic cytoplasm and numerous vacuoles resembling Burkitt cells. Chromosomal analysis revealed a complex chromosomal karyotype, including t(14;18)(q32;q21), and FISH analysis confirmed split BCL2, BCL6, and MYC signals. Bone marrow biopsy revealed diffusely infiltrating large abnormal lymphoid cells with a CD10⁺, CD20⁺, BCL2⁺, BCL6⁺, c-MYC⁺ and MUM1(-) immunophenotype. B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma and BL, was diagnosed. The patient achieved a partial response after eight courses of DA-EPOCH-R chemotherapy. Our experience suggests that DA-EPOCH-R may be an effective treatment for DHL/THL.
authors.affiliation: Department of Hematology and Oncology, Oji General Hospital.
authors.forename: Akari
authors.initials: A
authors.lastname: Hashimoto
authors.forename: Akihito
authors.initials: A
authors.lastname: Fujimi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kanisawa
authors.forename: Chisa
authors.initials: C
authors.lastname: Nakajima
authors.forename: Naotaka
authors.initials: N
authors.lastname: Hayasaka
authors.forename: Shota
authors.initials: S
authors.lastname: Yamada
authors.forename: Toshinori
authors.initials: T
authors.lastname: Okuda
authors.forename: Shinya
authors.initials: S
authors.lastname: Minami
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Takanori
authors.initials: T
authors.lastname: Shibata
authors.forename: Kota
authors.initials: K
authors.lastname: Hamaguchi
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Kamihara
authors.forename: Sari
authors.initials: S
authors.lastname: Iwasaki
authors.forename: Junji
authors.initials: J
authors.lastname: Kato
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D020732:Cytogenetic Analysis; D004268:DNA-Binding Proteins / Q000235:genetics; D004317:Doxorubicin / Q000008:administration & dosage / Q000627:therapeutic use; D005047:Etoposide / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics*; D008297:Male; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics; D014178:Translocation, Genetic; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: C000595707:BCL2 protein, human; C084103:BCL6 protein, human; D004268:DNA-Binding Proteins; C489427:MYC protein, human; D019253:Proto-Oncogene Proteins c-bcl-2; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone