International Prognostic Index

Several investigators have described the clinical aggressiveness of various subgroups of T-cell lymphomas and have correlated disease behavior with various factors at presentation. In the Non-Hodgkin's Lymphoma Classification Project analysis, there was a wide spectrum of clinical behavior for different T-cell lymphoma subsets (Table 60.2). Patients with anaplastic large T-cell lymphomas have the best 5-year survival rate (77%), while those with other T-cell subsets such as PTCL have much worse outcomes (25% 5-year survival).75 In a recent analysis of the 96 non-ALCL PTCL cases diagnosed within the Non Hodgkin's Lymphoma Classification Project, the frequency of PTCL varied from 1.5 to 18.3% according to country of origin. Seventy percent of the patients in this study had received combination chemotherapy which contained doxorubicin, resulting in 5-year failure-free and overall survival rates of 20 and 26%, respectively. Performance status and International Prognostic Index (IPI) scores at presentation strongly correlated with 5-year overall and failure-free survival results. However, other components of the IPI, such as age, clinical stage, serum lactate dehydrogenase (LDH), and the presence of extranodal involvement, were not statistically correlated with disease outcome. There were no statistically significant differences for the overall and failure-free survival rates among various major T-cell subtypes. Of note is the fact that within the PTCL, "not-otherwise-

Table 60.2 REAL classification of lymphoma entities according to clinical aggressiveness

5-Year overall survival rate Subgroup (%)

Anaplastic large T/null cell

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