Enteropathy associated T cell lymphoma with Reed-Sternberg-like cells of B cell phenotype and genotype associated with Epstein-Barr virus infection
We describe a case of a 76-year-old Caucasian male who presented to the Emergency Department (ED) with weight loss, abdominal pain, and multiple episodes of vomiting and diarrhea. He underwent a laparotomy with an intraluminal mass seen in the cecum. Histology showed atypical intermediate- to large-sized cells involving the full thickness of the bowel wall with numerous mitotic figures and apoptotic bodies. The adjacent uninvolved mucosa demonstrated villous blunting, increased intraepithelial lymphocytes, crypt elongation, and lamina propria plasmacytosis, consistent with the celiac enteropathy. Rare large transformed cells morphologically consistent with Reed-Sternberg cells (RS cells) were also identified in the mesenteric lymph nodes. Immunophenotyping showed the intermediate- to large-sized cells to be of T cell origin with strongly CD3, CD45, and TIA-1 positive; moderately positive for CD5; and variably positive for CD2, CD25, CD57, perforin, and granzyme B, with an aberrant loss of CD7. The large cells were moderately positive for PAX-5, CD79a, CD20, CD30, and MUM1. They also were positive for EBV latent membrane protein (EBV-LMP) and Epstein-Barr virus-encoded small RNA (EBER). Based on morphology and immunoprofile, a diagnosis of enteropathy associated T cell lymphoma (EATL) with Reed-Sternberg-like cells associated with EBV virus infection was made. This is a rare phenomenon; the presence of large B cell proliferations in EATL, to our knowledge, is yet to be reporte...
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Abstract Celiac disease (CD) is the most common autoimmune enteropathy worldwide. In CD, dietary gluten triggers a T cell driven small intestinal inflammation in a subset of genetically predisposed subjects, expressing the HLA DQ2 and/or DQ8 genes on their antigen presenting cells. HLA DQ2/DQ8 can bind gluten peptides after their prior modification by the CD autoantigen, tissue transglutaminase (TG2). This process leads to the activation of gluten reactive T cells, small bowel villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis, the histological hallmarks of CD. The clinical picture of CD is extre...