title: Clinicopathological features of double-hit B-cell lymphomas with MYC and BCL2, BCL6 or CCND1 rearrangements.
abstract: Double-hit (DH) lymphomas are B-cell lymphomas characterized by chromosomal rearrangements, specifically of MYC and either BCL2, BCL6 or CCND1. We reviewed 22 cases of DH lymphomas. BCL2/MYC DH lymphomas constituted the majority of these DH lymphomas (17 cases; 77%), followed by BCL6/MYC (2 cases; 9%) lymphomas. Assessing morphological features using the 2008 World Health Organization classification system, 15 cases (68%) were determined to be B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BCLU) (10 cases; 45%), or as DLBCL (5 cases; 23%), and 2 cases (9%) were classified as morphologically untransformed follicular lymphoma. Burkitt lymphoma was rare (1 case; 5%) among DH lymphomas. Nineteen cases were treated with R-CHOP or a high dose chemotherapy regimen. After a median follow-up of 11 months, 7 patients had died, and the 1-year survival rate was 62.5%. High dose chemotherapy did not improve the outcome. We suggest that screening of genetic variations to detect DH lymphomas is required in diagnosing all lymphomas, even those determined morphologically to be follicular lymphoma.
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Maki
authors.initials: M
authors.lastname: Yoshida
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Ayako
authors.initials: A
authors.lastname: Ichikawa
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Miyoshi
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Kiyasu
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Yoshizo
authors.initials: Y
authors.lastname: Kimura
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Fumiko
authors.initials: F
authors.lastname: Arakawa
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Niino
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D019938:Cyclin D1 / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D017404:In Situ Hybridization, Fluorescence / Q000379:methods; D016393:Lymphoma, B-Cell / Q000235:genetics* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; 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
chemical_list: C000595707:BCL2 protein, human; C084103:BCL6 protein, human; C530104:CCND1 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; D019938:Cyclin D1
keywords: B-cell lymphoma; BCL2; MYC; double-hit lymphoma; follicular lymphoma; unclassifiable with features intermediate between DLBCL and Burkitt lymphoma