Enteropathy-Type T-Cell Lymphoma: Clinical Features of a Series of 62 Cases. Jan Delabie, Harald Holte, Andrew Lister and Julie Vose for the International T-Cell Lymphoma Project.

In an international retrospective clinical study of 1159 adult, non-cutaneous mature T-cell lymphomas, verified by an expert pathology panel, 62 cases of enteropathy-type T-cell lymphoma (ETTCL) were identified, representing 5.3%. Patients in this study were diagnosed at one of the participating centers in North America, Europe, and Asia between 1990 and 2002. ETTCL represented the 6^th most common T-cell lymphoma, affecting predominantly the small bowel. Of interest, about a third of the cases of ETTCL were obtained from Norway, as the only participating center from Scandinavia. Twenty out of 52 cases (38%) were associated with celiac disease. As expected, abdominal pain was the most frequently recorded symptom, followed by general symptoms such as fatigue, weakness and anorexia. Overall as well as failure-free 5-year survival were poor, 20% and 4%, respectively. Univariate statistical analysis revealed that high T-IPI score (Gallamini, et al: Blood 103: 2474–2479, 2004) but not high IPI score, high CRP and LDH levels, and tumor size >5cm were adverse prognostic factors at p values <0.05. Most patients (51/60) were treated with anthracycline-containing chemotherapy regimens, whereas 9 received other or no treatment and showed a worse survival, probably due to a negative patient selection. Multivariate analysis controlling for T-IPI score, revealed that only high CRP levels and tumor size >5cm were independent prognostic factors. In conclusion, ETTCL is a clinically aggressive and fatal disease for which, currently, no effective treatment exists. T-IPI score, CRP levels and tumor size can be used as prognostic factors.