title: T-cell-rich B-cell lymphoma - diagnostic and therapeutic aspects.
abstract: BACKGROUND
  Morphologically, T-cell-rich B-cell lymphoma (TCRB-NHL) may be indistinguishable from Hodgkin's disease (HD). Immunophenotyping may be helpful in the separation of these entities. TCRB-NHL is occasionally misdiagnosed and treated as HD. However, information is limited regarding clinical characteristics and outcome of this patient population. Furthermore, knowledge concerning any association with Epstein-Barr virus (EBV) in TCRB-NHL, as well as the immunophenotype of reactive T-cells and the expression of T-cell intracellular antigen-1 (TIA-1), granzyme B (GrB) and the CD3-zeta-chain is limited.
  
  
  METHODS
  We have re-evaluated 251 tumour biopsies from patients aged > or =15 years with HD diagnosed 1985-1994. Reclassification from HD to TCRB-NHL was done in 12 cases (5%). Six TCRB-NHL patients initially diagnosed and treated as B-NHL were also included. All TCRB-NHL biopsies were analysed for latent membrane protein 1 (LMP-1), CD4, CD8, CD56, CD57, TIA-1, GrB and CD3-zeta-chain.
  
  
  RESULTS
  Twelve cases of TCRB-NHL were initially subclassified as HD (lymphocyte predominance 5, nodular sclerosis 3, and mixed cellularity 4). Of these 12 TCRB-NHL patients, 6 were given radiotherapy alone, 5 MOPP/ABVD or similar combination chemotherapy, and one patient combined modality treatment. Male sex (p<0.05) and inguinal involvement (p<0.001) were significantly more frequent when TCRB-NHL patients receiving HD treatment (n=12) were compared with the remaining patients with confirmed (conf) HD, while no significant differences were seen with regard to stage, bone marrow infiltration, splenomegaly or cause-specific survival. Similar results were achieved when all TCRB-NHL patients (n=18) were compared to conf HD patients. Lymphoma cells in three samples stained positively for LMP-1. A decreased expression of CD3-zeta-chain was seen in 9/14 tumour biopsies.
  
  
  CONCLUSIONS
  Immunohistochemistry makes it possible to identify cases of TCRB-NHL that are morphologically difficult to distinguish from HD. The outcome of TCRB-NHL patients treated as having HD was comparable with that of the remaining HD population.
authors.affiliation: Division of Hematology, Department of Medicine, Karolinska Hospital and Institutet, Stockholm, Sweden. ulla.axdorph@ks.se
authors.forename: Ulla
authors.initials: U
authors.lastname: Axdorph
authors.forename: Anna
authors.initials: A
authors.lastname: Porwit-Macdonald
authors.forename: Jan
authors.initials: J
authors.lastname: Sjøberg
authors.forename: Gunnar
authors.initials: G
authors.lastname: Grimfors
authors.forename: Magnus
authors.initials: M
authors.lastname: Bjørkholm
mesh_terms: D048868:Adaptor Proteins, Signal Transducing; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D001706:Biopsy; D002352:Carrier Proteins / Q000378:metabolism; D003598:Cytoskeletal Proteins; D003937:Diagnosis, Differential; D005260:Female; D053804:Granzymes; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping / Q000379:methods*; D047908:Intracellular Signaling Peptides and Proteins; D060588:LIM Domain Proteins; D016246:Lymphocytes, Tumor-Infiltrating / Q000276:immunology; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D008875:Middle Aged; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins / Q000378:metabolism; D012189:Retrospective Studies; D012697:Serine Endopeptidases / Q000378:metabolism; D000074019:T-Cell Intracellular Antigen-1; D013601:T-Lymphocytes / Q000166:cytology* / Q000276:immunology
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D015703:Antigens, CD; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D047908:Intracellular Signaling Peptides and Proteins; D060588:LIM Domain Proteins; D008565:Membrane Proteins; C484380:PDLIM7 protein, human; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases