Enteropathy-associated T-cell lymphoma

Abstract

Enteropathy-Associated T-Cell Lymphoma (EATL) is an extranodal peripheral T-cell lymphoma derived from intraepithelial lymphocytes (IELs) of the intestinal mucosa that commonly involves the small intestine and is classically associated with celiac sprue. EATLs, classified by the World Health Organization (WHO) into two subtypes with distinct clinical and pathologic features, are aggressive lymphomas with a dismal prognosis. Disease-and treatment-related complications, along with chemorefractory disease, are common. Therefore, the median overall survival is less than one year, with few long-term survivors. Improved understanding of the molecular pathogenesis of EATL is greatly needed if novel therapeutic approaches are to be developed for this aggressive and poorly understood T-cell lymphoma. In this chapter we will review the natural history, diagnosis, risk-stratification, and treatment strategies for EATL. © 2012 by Nova Science Publishers, Inc. All rights reserved.