title: Large B cell lymphoma presenting initially in bone marrow, liver and spleen: an aggressive entity associated frequently with haemophagocytic syndrome.
abstract: OBJECTIVE
  To describe diffuse large B cell lymphoma (DLBCL) presenting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy.
  
  
  RESULTS
  The clinicopathological and cytogenetic features of 11 such cases (eight men, three women; mean age: 62.7 years are described). Usually presenting with fever and haemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumour cells without sinusoidal involvement. All cases had a high Ki-67 index (≥90%), commonly a non-germinal centre/activated B cell immunophenotype and were negative for Epstein-Barr virus and human herpesvirus 6 and 8. The more frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18 and add(19)(p13). Clinical behaviour was very aggressive, with a 2-year survival rate of 18% (45% of patients died within 3 weeks). High-dose chemotherapy with haematopoietic stem cell transplantation prolonged survival in one patient.
  
  
  CONCLUSIONS
  Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.
authors.affiliation: Department of Internal Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
authors.forename: Yu-Min
authors.initials: YM
authors.lastname: Yeh
authors.forename: Kung-Chao
authors.initials: KC
authors.lastname: Chang
authors.forename: Ya-Ping
authors.initials: YP
authors.lastname: Chen
authors.forename: Lin-Yuan
authors.initials: LY
authors.lastname: Kao
authors.forename: Huey-Pin
authors.initials: HP
authors.lastname: Tsai
authors.forename: Chung-Liang
authors.initials: CL
authors.lastname: Ho
authors.forename: Jen-Ren
authors.initials: JR
authors.lastname: Wang
authors.forename: Dan
authors.initials: D
authors.lastname: Jones
authors.forename: Tsai-Yun
authors.initials: TY
authors.lastname: Chen
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology*; D002869:Chromosome Aberrations; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D003582:Cytogenetics; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000473:pathology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013154:Spleen / Q000473:pathology*