title: B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and burkitt lymphoma: study of 39 cases.
abstract: B-cell lymphoma, unclassifiable (B-UCL), with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma, is a poorly characterized entity. Therefore, we investigated cases of B-UCL treated by the Nebraska Lymphoma Study Group (NLSG). We searched the NLSG registry for years 1985-2010 for cases of B-UCL. Immunohistochemical stains and fluorescence in situ hybridization studies for MYC, BCL2 and BCL6 gene rearrangements were performed. Among the 39 cases studied, 54% were male and 46% were female, with a median age of 69 years. The majority of patients presented with advanced-stage disease (62%) and had high (3-5) International Prognostic Index (IPI) scores (54%). The median overall survival (OS) was only 9 months and the 5-year OS was 30%. Patients with low IPI scores (0-2) had a better survival than those with high scores (3-5). The cases were genetically heterogeneous and included 11 'double-hit' lymphomas with rearrangements of both MYC and BCL2 or BCL6. None of the immunohistochemical or genetic features was predictive of survival. This B-cell lymphoma is a morphologically-recognizable entity with a spectrum of genetic abnormalities. New and better treatments are needed for this aggressive lymphoma.
authors.affiliation: Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, Nebraska, USA.
authors.forename: Anamarija M
authors.initials: AM
authors.lastname: Perry
authors.forename: David
authors.initials: D
authors.lastname: Crockett
authors.forename: Bhavana J
authors.initials: BJ
authors.lastname: Dave
authors.forename: Pamela
authors.initials: P
authors.lastname: Althof
authors.forename: Lisa
authors.initials: L
authors.lastname: Winkler
authors.forename: Lynette M
authors.initials: LM
authors.lastname: Smith
authors.forename: Patricia
authors.initials: P
authors.lastname: Aoun
authors.forename: Wing C
authors.initials: WC
authors.lastname: Chan
authors.forename: Kai
authors.initials: K
authors.lastname: Fu
authors.forename: Timothy C
authors.initials: TC
authors.lastname: Greiner
authors.forename: Phillip
authors.initials: P
authors.lastname: Bierman
authors.forename: Robert
authors.initials: R
authors.lastname: Gregory Bociek
authors.forename: Julie M
authors.initials: JM
authors.lastname: Vose
authors.forename: James O
authors.initials: JO
authors.lastname: Armitage
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Weisenburger
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D002051:Burkitt Lymphoma / Q000175:diagnosis* / Q000628:therapy; D003131:Combined Modality Therapy; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor