C0647 - Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Tough Battle to Win!!

Kiran U. Revankar, MBBS, MD¹, Roy Mukkada, MBBS, MD, DNB², Abraham Koshy, MBBS, MD, DM², Pushpa Mahadevan, MBBS MD², Pradeep G. Mathew, MBBS, MD², Thara Pratap, MBBS, MD, DMRD, DNB², Antony Chettupuzha, MBBS, MD, DM², Maya Peethambaran, MBBS, MD, DNB, DNB, MRCPCH, MRCP, PGDAP², Dr. Shelly Paul, MBBS, MD, DM, PhD², Rasmi Unnikrishnan, MBBS, MD¹, Jerry Abraham Joseph, MD², Sreevidya Pillai, MBBS, MD², Hari Mangalath, MBBS, DNB², Ebin Thomas, MBBS, DNB², Khushboo Malhotra, MBBS, MS, DNB³, Jerlin Mathew, MBBS^2
1VPS Lakeshore Hospital and Research Centre, Kochi, Kerala, India; ²VPS Lakeshore Hospital, Kochi, Kerala, India; ³Amrita Hospital, Kochi, Kerala, India

Introduction: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as type 2 enteropathy-associated T-cell lymphoma (EATL), is an extremely rare and rapidly progressive type of non-Hodgkin’s lymphoma without an established targeted therapy at present. Our purpose is to report the diagnostic and therapeutic challenges we faced in treating this condition associated with high mortality.

Case Description/Methods: A 36-year-old gentleman presented to us with a history of chronic small bowel type of diarrhoea for 3 months associated with significant weight loss and high-grade fever. Baseline investigations including the stool examination were normal except for elevated C-reactive protein. Since oesophagogastroduodenoscopy (OGD) and colonoscopy showed normal mucosal patterns, CT enterography was done to evaluate the small bowel which revealed jejunal thickening and mesenteric lymphadenopathy. Single balloon enteroscopy showed multiple ulcerations in the proximal and mid jejunum involving more than 50% of the circumference with necrotic base. PET CT demonstrated significant diffuse uptakes in proximal jejunum, spleen, paraaortic lymph nodes, and axial skeleton. Biopsy from jejunal ulcers revealed surface erosions and distorted crypt-villous architecture with dense infiltration by the monotonous population of medium-sized lymphoid cells with scanty cytoplasm and round hyperchromatic nuclei. Multiple atypical lymphoid cells and scattered mitotic figures were seen. These atypical lymphoid cells were positive for CD3, bcl2, CD8, and CD56 with a high MIB index (60 - 70%) and were negative for CD4 and CD30. No CD20 positive B cells were seen among the infiltrating cells. Immunohistochemistry (IHC) was consistent with MEITL. Tissue transglutaminase IgA antibody test was done to screen for celiac disease which was negative. The patient is on chemotherapy (CHOP Regimen) but with an unsatisfactory response at present.

Discussion: MEITL is a rare type of primary T cell lymphoma with an aggressive clinical course often presenting at an advanced stage of the disease. MEITL was reclassified from EATL in the 2016 WHO classification because of the lack of association with celiac disease. Small bowel evaluation and biopsy with IHC are the cornerstones of the diagnosis. Though there is no established therapy yet, the literature on stem cell transplantation has shown promising results.



Disclosures:


Kiran U. Revankar, MBBS, MD¹, Roy Mukkada, MBBS, MD, DNB², Abraham Koshy, MBBS, MD, DM², Pushpa Mahadevan, MBBS MD², Pradeep G. Mathew, MBBS, MD², Thara Pratap, MBBS, MD, DMRD, DNB², Antony Chettupuzha, MBBS, MD, DM², Maya Peethambaran, MBBS, MD, DNB, DNB, MRCPCH, MRCP, PGDAP², Dr. Shelly Paul, MBBS, MD, DM, PhD², Rasmi Unnikrishnan, MBBS, MD¹, Jerry Abraham Joseph, MD², Sreevidya Pillai, MBBS, MD², Hari Mangalath, MBBS, DNB², Ebin Thomas, MBBS, DNB², Khushboo Malhotra, MBBS, MS, DNB³, Jerlin Mathew, MBBS². C0647 - Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Tough Battle to Win!!, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.