Case Presentation: A 67 year old woman presented with right upper quadrant abdominal pain and was diagnosed with acute cholecystitis. She underwent elective cholecystectomy, complicated by Clostridium difficile colitis. Despite treatment with oral Vancomycin, she had persistent diarrhea and failure to thrive for a month. Repeat Clostridium difficile toxin was negative. Esophagogastroduodenoscopy showed erosive gastritis and normal duodenal mucosa with biopsy showing chronic duodenitis and villous atrophy. Immunohistochemistry demonstrated increased number of lymphocytes on CD8 and CD3 stains within surface epithelium and within the crypts. Tissue Transglutaminase Antibody IgA (TTGA) was positive.
Despite being on gluten-free diet for celiac disease, she returned with watery diarrhea, severe abdominal pain and hemoccult positive stools. CT scan demonstrated spontaneous perforation of mid-jejunum and multiple “lesions” in jejunum. She underwent small bowel resection and primary anastomosis. Intra-operatively, lesions were noted along the jejunum with desmoplastic reaction on the surface, with thickened mesentery and enlarged mesenteric lymph nodes. Patient became hemodynamically unstable requiring pressor support, followed by multi-organ failure.
Due to rapid deterioration, family made patient comfort measures only and patient passed away soon after. Later, immunohistochemistry of jejunal specimens returned positive for CD3, CD43, bcl-2 and Ki-67 (60%) expression and negative for CD5, all consistent with EATL.
Discussion: EATL accounts for less than 5% GI lymphomas and less than 1% of all Non-Hodgkin lymphomas. Management of celiac disease at an early stage with strict gluten free diet adherence is associated with risk reduction of EATL.
Presentation range from malabsorption, diarrhea to acute abdominal pain arising from obstruction, perforation or bleeding. EATL mostly involves the small bowel and occasionally colon or stomach.
Intestinal findings include villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis invading the intestina wall at variable depths. Immunophenotype is CD3+, CD5-, CD4-, CD8+/-, CD30+, CD56-, TCR beta +/- and CD 103+ and its associated to positive TTGA, IgA anti-endomysial or IgG Deamidated Gliadin Peptide.
There is no standard of care for newly diagnosed EATL. Multimodality approach is warranted based on clinical scenario including combination chemotherapy, surgery and autologous stem cell transplantation. Targeted therapies, like JAK/STAT inhibitors and Brentuximab vedotin (anti-CD30) are being investigated.
Conclusions: EATL is a rare, aggressive gastrointestinal non-Hodgkin’s lymphoma arising from intraepithelial intestinal Tlymphocytes. It is strongly associated with celiac disease and is diagnosed in its terminal stages. EATL should be suspected in a patient with celiac disease with abrupt worsening of abdominal pain, diarrhea and failure to thrive. More investigations for newer targeted therapies are needed for this rare fatal disease.