MONOMORPHIC EPITHELIOTROPIC INTESTINAL T-LYMPHOMA – CASE REPORT

Background: Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) (formerly termed enteropathy-associated T cell lymphoma, type II) is an extremely rare peripheral T-cell lymphoma that involves the malignant proliferation of a T- lymphocyte in the gastrointestinal tract. Over time, these T cells commonly spread throughout the mucosal lining of a portion of the GI tract (particularly the jejunum and ileum of the small intestine), lead to GI tract nodules and ulcerations, and cause symptoms such as abdominal pain, weight loss, diarrhea, obstruction, bleeding, and/or perforation. Its clinical, morphologic, and immunophenotypic features distinguishing it from the more common Enteropathy Associated T-Lymphoma (previously EATL type I) made it a separate entity. Case report: We present a case of a rare extremely aggressive T cell lymphoma that originates from the gastrointestinal tract, spreads to surrounding organs and is refractory to surgery and chemotherapy. We describe а case report of 79 years old patient with clinical features of acute abdomen, ileus, treated with surgery two times. During the first surgery partial resection of jejunum was performed. Pathohistology and immunоhistochemistry findings of MEITL. PET/CT scan revealed infiltration of intestinum, uterus, ovarium. During the second operation partial resection of ileum and hysterectomy with adnexectomy was performed. The patient is treated with antracycline-based regimen CHOP21 (4 cycles). Re-evaluation with second PET/CT scan revealed residual tumor on the intestinum and bladder. The patient deteriorate with acute renal failure and multi-organ failure. Despite aggressive treatment with extensive surgery and aggressive anthracycline-based chemotherapy, in a short time the tumor spread to surrounding organs (sigma, bladder). The patient survived 11 months from the initial definitive diagnosis. Conclusion: MEITL is a challenging primary intestinal T cell lymphoma to treat as the outcome is frequently poor despite surgery and chemotherapy. Most patients are elderly with co-morbidities and they usually present late rendering any therapy ineffective. Young age, early Ann-Arbor/Lugano disease stage, good performance scale status, patients receiving autologous stem cell transplantation and less bulky disease are associated with an improved survival outcome. Further research is needed to incorporate new therapeutic modalities based on molecular research for successful treatment of this aggressive lymphoma.