Multiple cerebral lesions in a patient with refractory celiac disease:A case report  

作　　者：Lena Horvath Georg Oberhuber Andreas Chott Maria Effenberger Herbert Tilg Eberhard Gunsilius Dominik Wolf Sarah Iglseder 

机构地区：[1]Department of Internal Medicine V(Hematology and Medical Oncology),Medical University Innsbruck,Innsbruck 6020,Austria [2]InnPath GmbH,Institute of Pathology,Innsbruck 6020,Austria [3]Ottakring Clinic,Institute of Pathology and Microbiology,Vienna 1160,Austria [4]Department of Internal Medicine I(Gastroenterology,Hepatology,Endocrinology and Metabolism),Medical University Innsbruck,Innsbruck 6020,Austria [5]Department of Neurology,Medical University Innsbruck,Innsbruck 6020,Austria

出　　处：《World Journal of Gastroenterology》2020年第47期7584-7592,共9页世界胃肠病学杂志（英文版）

摘　　要：BACKGROUND 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 predominantly 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).CASE SUMMARY 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.18Ffluorodeoxyglucose-positron emission tomography/computed tomography(18FDG-PET/CT)scan showed a suspect hypermetabolic lesion in the left upper abdomen and consequent surgical jejunal resection revealed the diagnosis of EATL.During the diagnostic work-up,neurological symptoms aggravated and evolved refractory to high-dosage cortisone.Recurrent MRI scans showed progressive cerebral lesions,highly suspicious for lymphoma and methotrexate chemotherapy was initiated.Unfortunately,clinically the patient responded only transiently.Finally,cerebral biopsy confirmed the diagnosis of cerebral involvement of EATL.Considering the poor prognosis and deterioration of the performance status,best supportive care was started.The patient passed away three weeks after diagnosis.CONCLUSION EATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.

关 键 词：Enteropathy-associated T cell lymphoma Brain neoplasm Celiac disease Cerebellar syndrome Case report 

分 类 号：R735.3[医药卫生—肿瘤]