Enteropathy-associated T-cell lymphoma (EATL) is a rare subtype of non-Hodgkin's lymphoma characterized by an aggressive phenotype and poor outcome in the vast majority of cases. Substantial portions of patients have either no prior diagnosis of celiac disease or have a subacute course of celiac disease followed by EATL diagnosis. We report a case of a 72-year-old African American male without history of gastrointestinal intolerance who presented with acute abdominal pain and weight loss leading to the eventual diagnosis of gastric EATL. Despite an initial promising clinical and radiographic response to cyclophosphamide, doxorubicin, vincristine, and prednisone plus alemtuzumab, the disease rapidly progressed with a fatal outcome. This case and review of the literature highlights the features of this uncommon disease and addresses both the diagnostic and therapeutic challenges of this aggressive malignancy. We also discuss our experience with the use of fluoro-2-deoxy-d-glucose positron emission tomography in monitoring treatment response.
- Celiac disease
- EATL (enteropathy-associated T-cell lymphoma)
ASJC Scopus subject areas