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T-cell-rich B-cell lymphoma - diagnostic and therapeutic aspects
Ulla Axdorph1, Anna Porwit-Macdonald, Jan Sjøberg
1Division of Hematology, Department of Medicine, Karolinska Hospital and Institutet, Stockholm, Sweden. ulla.axdorph@ks.se
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.
Patients And 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.
Conclusion:
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.