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B ‐cell lymphoma, unclassifiable, with features intermediate between diffuse large B ‐cell lymphoma and burkitt lymphoma: study of 39 cases
Author(s) -
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.
Publication year - 2013
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12343
Subject(s) - bcl6 , lymphoma , medicine , international prognostic index , diffuse large b cell lymphoma , immunohistochemistry , fluorescence in situ hybridization , pathology , stage (stratigraphy) , b cell lymphoma , oncology , b cell , gastroenterology , immunology , biology , gene , antibody , chromosome , germinal center , paleontology , biochemistry
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 , BCL 2 and BCL 6 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 BCL 2 or BCL 6 . 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.