Perforation of the small intestine caused by enteropathyassociated T cell lymphoma

Özet

Enteropathy-associated T cell lymphoma is a rare lymphoma specific to the gastrointestinal system, arising fromintraepithelial T lymphocytes, that is often associated with celiac disease. We report a 53-year-old female patientwith no previous disease who presented with severe abdominal pain. Physical examination revealed diffuse abdominal tenderness and abdominal guarding and the patient underwent emergency surgery with a diagnosis ofacute abdomen. During the operation, a 20-cm mass was found located on Treitz ligament, invading the duodenumand pancreatic head and perforating the jejunum. Histologically, medium-sized monomorphic atypical lymphocyteinfiltration with dark nucleus and narrow cytoplasm was seen in the layers of mucosa, submucosa, muscular wall,and serosa of the duodenum. The final pathological diagnosis was “enteropathy-associated T cell lymphoma type2” based on immunohistochemical and serological findings. Based on the World Health Organization 2008 criteria,enteropathy-associated T cell lymphoma has two subtypes. Type 1 enteropathy-associated T cell lymphoma is associated with celiac disease and has HLA DQ2 and HLA DQ8 genotype. Enteropathy-associated T cell lymphoma 2enteropathy-associated T cell lymphoma seldom occurs and is not associated with celiac disease.