B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and burkitt lymphoma: Study of 39 cases

Anamarija M. Perry, David Crockett, Bhavana J. Dave, Pamela Althof, Lisa Winkler, Lynette M. Smith, Patricia Aoun, Wing C. Chan, Kai Fu, Timothy C. Greiner, Phillip Bierman, Robert Gregory Bociek, Julie M. Vose, James O. Armitage, Dennis D. Weisenburger

Research output: Contribution to journalArticle

53 Scopus citations

Abstract

Summary: 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.

Original languageEnglish (US)
Pages (from-to)40-49
Number of pages10
JournalBritish Journal of Haematology
Volume162
Issue number1
DOIs
StatePublished - Jul 2013

Keywords

  • BCL2
  • Burkitt lymphoma
  • Diffuse large B-cell lymphoma
  • MYC
  • Unclassifiable B-cell lymphoma

ASJC Scopus subject areas

  • Hematology

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