Published online Dec 21, 2020. doi: 10.3748/wjg.v26.i47.7584
Peer-review started: August 24, 2020
First decision: October 18, 2020
Revised: October 31, 2020
Accepted: November 12, 2020
Article in press: November 12, 2020
Published online: December 21, 2020
Enteropathy-associated T cell lymphoma (EATL) is an aggressive intestinal T cell lymphoma derived from intraepithelial lymphocytes, which occurs in individuals with celiac disease (CD). Cerebral involvement is an extremely rare condition and as described so far, lymphoma lesions may present as parenchymal predo-minantly supratentorial or leptomeningeal involvement. We describe a case of EATL with multifocal supra- and infratentorial brain involvement in a patient with refractory celiac disease (RCD).
A 58-years old man with known CD developed ulcerative jejunitis and was diagnosed with RCD type II. Six months later he presented with subacute cerebellar symptoms (gait ataxia, double vision, dizziness). Cranial magnetic resonance imaging (MRI) revealed multifocal T2 hyperintense supra- and infratentorial lesions. Laboratory studies of blood and cerebrospinal fluid were inconspicuous for infectious, inflammatory or autoimmune diseases. 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (
EATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.
Core Tip: Enteropathy-associated T cell lymphoma (EATL) is a rare type of peripheral T cell lymphoma frequently evolving from refractory celiac disease (RCD) type II. The prognosis is often dismal due to its aggressive clinical behavior. Extraintestinal manifestation may occur, but concerning cerebral involvement, only very few reports have been described so far, showing parenchymal lesions with predominantly supratentorial or leptomeningeal involvement. This case shows multifocal supratentorial, brainstem and cerebellar involvement of EATL in a patient with RCD type II, with a rarely observed gamma/delta T cell receptor immunophenotype. This report underlines the importance to consider EATL with cerebral involvement in patients with RCD who develop neurological symptoms.