Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Extraintestinal Areas of Peripheral T-Cell Lymphoma Involvement

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a primary intestinal T-cell lymphoma, previously known as enteropathy-associated T-cell lymphoma (EATL) type II. Its clinical, morphologic, and immunophenotypic features distinguishing it from the more common EATL (previously EATL type I) made it a separate entity. Unlike EATL, MEITL typically is noted in Asian, Hispanic, and indigenous populations; it is rarer in native European and Caucasian populations. Due to its poor prognosis, it needs to be distinguished from inflammatory diseases and less aggressive T-cell lymphomas. We present an unusual case of MEITL in a Caucasian patient who developed nonspecific GI symptoms and was diagnosed with MEITL of the jejunum, mesenteric lymph nodes, and multiple extraintestinal sites based on histology, immunophenotype, molecular testing, and imaging. Despite aggressive treatment, he expired about seven months after the definitive diagnosis.