International T-Cell Lymphoma Classification Project: Angioimmunoblastic T-Cell Lymphoma.

Peripheral T-cell lymphomas are rare diseases: therefore, the International T-cell Lymphoma Project was undertaken to compare lymphomas at different sites throughout North America, Asia and Europe. Within this project, 243 angioimmunoblastic T-cell lymphomas (AILTs) were diagnosed which made up 21% of all peripheral T-cell lymphoma (PTCL). At presentation, generalized lymphadenopathy was noted in 76% of the patients. Interestingly, three patients presented with extranodal disease only. Among the skin symptoms, erythroderma was the most frequent (21% of patients). Hemolytic anemia was seen in 13% and dysproteinemia occurred in 50%, and among these monoclonal serum immunoglobulin was seen in 8% of the patients. Anemia, hypergammaglobulinemia and elevated LDH were significantly more frequent in AILT than in PTCL-unspecified. Similarly, patients with AILT had a significantly higher frequency of high stage disease (89% of the patients were stage 3 or 4), as well as worse prognostic indices. Despite this, their 5-year overall (33%) and failure-free survivals (18%) were similar to patients with PTCL-unspecified. Treatment was usually administered in combination with anthracycline. A few factors at presentation were prognostic for outcome, including the PIT (prognostic index for T-cell lymphoma; Gallamini et al.: Blood. 2004 Apr 1;103(7):2474–9), age, B-symptoms and performance status. The IPI, however, was not prognostic. Controlling for the PIT, a platelet count <150.000 μl was prognostic for overall survival whereas B-symptoms were prognostic for failure-free survival. In conclusion, AILT is an aggressive disease for which the optimum treatment has not yet been developed.