Enteropathy-Associated T-Cell Lymphoma: Improving Treatment Strategies

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare and usually rapidly fatal intestinal T-cell non-Hodgkin lymphoma. It arises from intraepithelial lymphocytes and has a high association with coeliac disease. The high mortality of EATL is associated not only with the very aggressive and often chemotherapy-refractory nature of the lymphoma. The poor condition of patients due to prolonged and severe malnutrition compromises the ability to deliver chemotherapy. There are no standardized treatment protocols, and the optimal therapy for EATL remains unclear. The primary step of treatment consists of local debulking, preferably as early as possible after EATL diagnosis. Morbidity and mortality seem to rise with advanced stages of disease due to tumour size progression, worse nutritional status and a higher risk of emergency surgery due to perforation. Standard induction therapy for EATL is anthracycline-based chemotherapy, preferably resumed between 2 and 5 weeks after surgery (depending on clinical condition). Intensification of therapy using high-dose chemotherapy followed by consolidation with BEAM and autologous stem cell transplantation is associated with better outcome. Notably, this treatment strategy has only been applied in patients eligible for this aggressive regimen which might reflect selection bias. Unfortunately, prognosis of EATL remains poor; 5-year survival varies from 8 to 60% depending on the eligibility to receive additional steps of therapy. New treatment strategies are urgently needed for a better prognosis of this lethal complication of coeliac disease. Brentuximab vedotin (anti-CD30) might be promising when added to conventional chemotherapy and is suggested as upfront treatment in EATL.

Gastrointestinal lymphoma: the new mimic

“…The prognosis for intestinal T-cell lymphomas, in general, and for EATL type I (ie, that associated with coeliac disease) and monomorphic epitheliotropic intestinal T-cell lymphoma (formerly referred to as EATL type II), in particular, is poor and management strategies for these and other rare forms of GI lymphoma vary according to presentation, location, stage and histological type and may involve chemotherapy, radiation therapy, immunotherapy, surgery and autologous stem cell transplantation 51–53. In one study which included a variety of peripheral T-cell lymphomas the response rate to cyclophosphamide-oncovin-prednisolone at a median follow-up of 27 months was 62% 54…”

Small‐bowel capsule endoscopy in patients with celiac disease, axial versus lateral/panoramic view: Results from a prospective randomized trial

“…Although CD usually responds well to a gluten-free diet (GFD) with an excellent prognosis, 1,2 a minority of patients can develop severe complications, such as type I or II refractory CD (RCDI, RCDII), ulcerative jejunoileitis (UJ) and malignancies of the gastrointestinal tract, such as enteropathyassociated T-cell lymphoma (EATL) and adenocarcinoma of the small bowel (SB). [3][4][5] In recent years, several diagnostic strategies have been proposed for early identification of these serious CD complications. 6 In particular, capsule enteroscopy (CE) has been proposed as a powerful tool to evaluate patients with persistent or new symptoms in spite of ongoing GFD.…”

Hematopoietic stem cell transplantation for T-cell lymphoma

“…These results might suggest that up‐front auto‐HSCT is a promising and curative treatment strategy for the patients with EATL, although these results were from subgroup analysis and there might be a selection bias. On the other hand, the information regarding allo‐HSCT for patients with EATL is limited …”