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

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 y...

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Veröffentlicht in:British journal of haematology 2013-07, Vol.162 (1), p.40-49
Hauptverfasser: 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.
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container_issue 1
container_start_page 40
container_title British journal of haematology
container_volume 162
creator 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.
description 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.
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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. 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Myelofibrosis ; Lymphoma, B-Cell - classification ; Lymphoma, B-Cell - diagnosis ; Lymphoma, B-Cell - therapy ; Lymphoma, Large B-Cell, Diffuse - diagnosis ; Lymphoma, Large B-Cell, Diffuse - therapy ; Male ; Medical sciences ; Middle Aged ; MYC ; Neoplasm Staging ; Treatment Outcome ; Tumors ; unclassifiable B‐cell lymphoma</subject><ispartof>British journal of haematology, 2013-07, Vol.162 (1), p.40-49</ispartof><rights>2013 John Wiley &amp; Sons Ltd</rights><rights>2014 INIST-CNRS</rights><rights>2013 John Wiley &amp; Sons Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4233-3764c85be43aa9b0b6a497f6b9793646370ae22f7bca4f307e0948aceee6e9f13</citedby><cites>FETCH-LOGICAL-c4233-3764c85be43aa9b0b6a497f6b9793646370ae22f7bca4f307e0948aceee6e9f13</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fbjh.12343$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fbjh.12343$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,1433,27924,27925,45574,45575,46409,46833</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=27469306$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23600716$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Perry, Anamarija M.</creatorcontrib><creatorcontrib>Crockett, David</creatorcontrib><creatorcontrib>Dave, Bhavana J.</creatorcontrib><creatorcontrib>Althof, Pamela</creatorcontrib><creatorcontrib>Winkler, Lisa</creatorcontrib><creatorcontrib>Smith, Lynette M.</creatorcontrib><creatorcontrib>Aoun, Patricia</creatorcontrib><creatorcontrib>Chan, Wing C.</creatorcontrib><creatorcontrib>Fu, Kai</creatorcontrib><creatorcontrib>Greiner, Timothy C.</creatorcontrib><creatorcontrib>Bierman, Phillip</creatorcontrib><creatorcontrib>Gregory Bociek, Robert</creatorcontrib><creatorcontrib>Vose, Julie M.</creatorcontrib><creatorcontrib>Armitage, James O.</creatorcontrib><creatorcontrib>Weisenburger, Dennis D.</creatorcontrib><title>B‐cell lymphoma, unclassifiable, with features intermediate between diffuse large B‐cell lymphoma and burkitt lymphoma: study of 39 cases</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>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. 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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.</abstract><cop>Oxford</cop><pub>Blackwell</pub><pmid>23600716</pmid><doi>10.1111/bjh.12343</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record>
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subjects Adult
Aged
BCL2
Biological and medical sciences
Biomarkers, Tumor - genetics
Biomarkers, Tumor - metabolism
Burkitt lymphoma
Burkitt Lymphoma - diagnosis
Burkitt Lymphoma - therapy
Combined Modality Therapy
Cytogenetic Analysis
Diagnosis, Differential
diffuse large B‐cell lymphoma
Female
Hematologic and hematopoietic diseases
Humans
Immunophenotyping
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphoma, B-Cell - classification
Lymphoma, B-Cell - diagnosis
Lymphoma, B-Cell - therapy
Lymphoma, Large B-Cell, Diffuse - diagnosis
Lymphoma, Large B-Cell, Diffuse - therapy
Male
Medical sciences
Middle Aged
MYC
Neoplasm Staging
Treatment Outcome
Tumors
unclassifiable B‐cell lymphoma
title B‐cell lymphoma, unclassifiable, with features intermediate between diffuse large B‐cell lymphoma and burkitt lymphoma: study of 39 cases
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