PERIPHERAL T-CELL LYMPHOMA - A CLINICOPATHOLOGICAL STUDY OF 41 CASES AND EVALUATION OF THE PROGNOSTIC-SIGNIFICANCE OF THE UPDATED KIEL CLASSIFICATION

A total of 41 non-cutaneous peripheral T-cell lymphomas were classifie d following the updated Kiel classification. Of these, 20 cases belong ed to the low-grade group (T-cell chronic lymphocytic leukaemia, 3: ly mphoepithelioid, 5; angioimmunoblastic, 4; pleomorphic small cell, 8) and 21 to the high grade group (pleomorphic medium and large cell, 11; immunoblastic, 3, large-cell anaplastic Ki-1 positive, 7). Seventy pe r cent showed a CD4 + /CD8-phenotype, 39% a defective phenotype and 88 % an activation phenotype. Eighty per cent had B-symptoms, 63% hepatom egaly, 48% splenomegaly and 26% had involvement of more than three lym phoid areas. Bone marrow was infiltrated in 34%, central nervous syste m in 4%, lung in 12% and skin in 14.6%. Seventeen per cent presented w ith extranodal disease and 82.8% had stage III/IV disease. Hypergammag lobulinaemia was found in 29%, hypercalcaemia in 7%, raised LDH serum levels in 58% and HTLV-1 antibodies in only one case. Of the 37 treate d patients 18 (48%) achieved a complete remission, but 33% relapsed. M ortality was 59% and actuarial overall survival at 38 months was 0.32. In the comparison of the clinical, analytical and immunophenotypic va riables and outcome between low and high grade groups, only the averag e of bone marrow infiltration in the low grade and stage I-II, presenc e of defective phenotypes and higher Ki-67 positivity in the high grad e group were significantly different. In the statistical studies, extr anodal presentation and the failure to achieve a complete remission we re the only variables that influenced mortality: there were no signifi cant differences in the general features of the low and high grade gro ups and only minor differences were found in the immunoblastic and ang ioimmunoblastic subgroups. There were no differences in the actuarial survival between the low and high grade groups, among the subgroups of the Kiel classification, among stages I to IV, between patients with or without B-symptoms, with or without defective phenotypes, Ki-67 pos itivity over or under 60%, or among different CD4/CD8 phenotypes. The updated Kiel classification did not separate groups with a prognostic significance.