title: Double-hit and double-protein-expression lymphomas: aggressive and refractory lymphomas.
abstract: Double-hit lymphoma (DHL) is a subgroup of aggressive lymphomas with both MYC and BCL2 gene rearrangements, characterised by a rapidly progressing clinical course that is refractory to aggressive treatment and short survival. Over time, the definition was modified and now includes diffuse large B-cell lymphoma (DLBCL) with MYC translocation combined with an additional translocation involving BCL2 or BCL6. Some cases that have a similar clinical course with concomitant overexpression of MYC or BCL2 proteins were recently characterised as immunohistochemical double-hit lymphomas (ie, double-protein-expression lymphomas [DPLs]). The clinical course of these DPLs is worse than so-called standard DLBCL but suggested by some studies to be slightly better than DHL, although there is overlap between the two categories. Present treatment does not allow cure or long-term survival in patients with genetic or immunohistochemical double-hit lymphomas, but several new drugs are being developed.
authors.affiliation: Haematology Department, Hospices Civils de Lyon, Lyon, France; University Lyon 1, Lyon, France.
authors.forename: Clémentine
authors.initials: C
authors.lastname: Sarkozy
authors.affiliation: Service d'Anatomie Pathologique, Hospices Civils de Lyon, Lyon, France; University Lyon 1, Lyon, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Traverse-Glehen
authors.affiliation: Haematology Department, Hospices Civils de Lyon, Lyon, France; University Lyon 1, Lyon, France. Electronic address: Bertrand.coiffier@chu-lyon.fr.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
mesh_terms: D004268:DNA-Binding Proteins / Q000235:genetics; D015972:Gene Expression Regulation, Neoplastic; D015321:Gene Rearrangement; D016259:Genes, myc / Q000235:genetics; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000473:pathology / Q000628:therapy; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics; D051560:Proto-Oncogene Proteins c-bcl-6
chemical_list: C000595707:BCL2 protein, human; C084103:BCL6 protein, human; D004268:DNA-Binding Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D051560:Proto-Oncogene Proteins c-bcl-6