B‐cell lymphoma, unclassifiable, with features intermediate between diffuse large B‐cell lymphoma and burkitt lymphoma: study of 39 cases. (18th April 2013)

• Record Type: Journal Article
• Authors: Perry, Anamarija M.; Crockett, David; Dave, Bhavana J.; Althof, Pamela; Winkler, Lisa; Smith, Lynette M.; Aoun, Patricia; Chan, Wing C.; Fu, Kai; Greiner, Timothy C.; Bierman, Phillip; Gregory Bociek, Robert; Vose, Julie M.; Armitage, James O.; Weisenburger, Dennis D.
• Is Part Of: British journal of haematology. Volume 162:Number 1(2013:Jul.)
• Page Start: 40
• Page End: 49
• Publication Date: 2013-04-18
• Subjects: Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.12343
• Languages: English
• ISSNs: 0007-1048

Abstract

<abstract abstract-type="main" xml:lang="en" id="bjh12343-abs-0001"> <title>Summary</title> <p>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 <italic>in situ</italic> hybridization studies for <italic>MYC, BCL2</italic> and <italic>BCL6</italic> 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 <italic>MYC</italic> and <italic>BCL2</italic> or <italic>BCL6</italic>. 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.</p> </abstract>