title: BCL-2 and MYC gain/amplification is correlated with central nervous system involvement in diffuse large B cell lymphoma at leukemic phase.
abstract: BACKGROUND
  Diffuse large B-cell lymphoma (DLBCL) of leukemic phase is a rare clinical manifestation, but is highly prevalent with central nervous system involvement (CNSI). Little is known about this rare clinical observation.
  
  
  METHODS
  We reviewed the clinical characteristics of 40 DLBCL patients with leukemic phase identified by flow cytometry and analyzed BCL2 and MYC aberrations by fluorescence in situ hybridization.
  
  
  RESULTS
  The median age of these 40 patients was 46 years (range, 15-75) with 19 men patients. All patients had bone marrow involvement, and fourteen (35.0%) had CNSI. There were respectively 14 patients (35.0%) had the BCL2 or MYC gain/amplification and nine of them (22.5%) simultaneously had both aberrations. Compared to those without CNSI, CNSI was found more commonly in male patients (71.4 vs. 34.6%, p = 0.046), in those with IPI scores of 4-5 (57.1% vs. 11.5%, p = 0.001), and in those with elevated serum LDH (100 vs. 61.5%, p = 0.007) and both MYC and BCL2 rearrangement (88.9 vs. 19.4%; p = 0.000). BCL2 and MYC rearrangements were the sole independent factor correlated with CNSI.
  
  
  CONCLUSIONS
  It is possible that both BCL2 and MYC gene aberrations may contribute to the high incidence of CNSI observed in leukemic phase of patients with DLBCL.
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Dehui
authors.initials: D
authors.lastname: Zou
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Shuhua
authors.initials: S
authors.lastname: Yi
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.|Department of Hematology, Tianjin First Center Hospital, Tianjin, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Cui
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Liu
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Chengwen
authors.initials: C
authors.lastname: Li
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Shizhen
authors.initials: S
authors.lastname: Zhong
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Zhen
authors.initials: Z
authors.lastname: Yu
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Zengjun
authors.initials: Z
authors.lastname: Li
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Lv
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Kun
authors.initials: K
authors.lastname: Ru
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Huijun
authors.initials: H
authors.lastname: Wang
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Gang
authors.initials: G
authors.lastname: An
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Xu
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.288, Nanjing road, Heping district, Tianjin, 300020, China. qiulg@ihcams.ac.cn.
authors.forename: Lugui
authors.initials: L
authors.lastname: Qiu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D002490:Central Nervous System / Q000378:metabolism; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007770:L-Lactate Dehydrogenase / Q000097:blood; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics*; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D000069283:Rituximab / Q000008:administration & dosage; D033581:Stem Cell Transplantation; D015996:Survival Rate; D014182:Transplantation, Autologous; D055815:Young Adult
chemical_list: D019253:Proto-Oncogene Proteins c-bcl-2; D016271:Proto-Oncogene Proteins c-myc; D000069283:Rituximab; D007770:L-Lactate Dehydrogenase
keywords: BCL2; Centre nervous system involvement; Diffuse large B cell lymphoma; Leukemic phase; MYC