Enteropathy-Associated T-Cell Lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a rare non-Hodgkin’s lymphoma involving the small bowel. Clinical features include gastrointestinal (GI) manifestations and systemic B symptoms. EATL risk factors include untreated celiac disease (CeD), gluten-free diet (GFD) nonadherence, and refractory celiac disease (RCD). Malignant T-lymphocytes are characterized by expression of CD3, CD7, and cytotoxic T-cell markers. Genetic alterations manifest as clonally rearranged T-cell receptor genes, gene amplifications, and mutations involved in oncogenic signaling pathways. Diagnosis of EATL requires evaluation of a tissue specimen, frequently acquired through advanced endoscopic or surgical techniques. Computerized tomography (CT) scans, magnetic resonance enterography (MRE), and/or positron emission tomography (PET) are needed to assess disease extent. Randomized trials guiding management are lacking, and clinical trial participation is preferred. Anthracycline-based chemotherapy followed by autologous stem cell transplantation (SCT) is commonly employed. Outcomes with this approach are suboptimal, and improved therapies are needed. Clinical trials evaluating novel strategies including antibody drug conjugates and immune-mediated strategies are underway.