Abstract
Background
The diagnosis of Crohn’s disease (CD) can sometimes be challenging. Enteropathy associated T cell lymphoma (EATL) is a rare aggressive lymphoma highly associated with celiac disease1. It usually affects small bowel, with signs and symptoms which may mimic CD.
Methods
We present a case of a 37-year-old male patient with coeliac disease, CD and (mis)diagnosed EATL in proximal jejunum.
Results
Patient was referred to our clinic with a constant epigastric pain, weight loss (BMI 17.5), intermittent temperatures, joint pain and diarrhoea. Laboratory tests were normal. Serology for coeliac disease was highly positive. Abdominal ultrasound showed small amount of ascites. EGD showed small shallow stomach ulcers and aphthous mucosal changes of postbulbar part of duodenum. Histology suggested coexisting CD and celiac disease. CT scan detected thickened duodenal wall and suspected duodenal stenosis and enteroscopy was performed. Approximately 80 cm from pyloric ring multiple jejunal ulcers were found. Unfortunately, biopsy was not performed. Colonoscopy showed small ulcers of terminal ileum and histology was nonspecific. Diagnosis of coexisting CD and coeliac disease was established. Therapy with PPI, systemic glucocorticoids and azatioprine was introduced. Three months later patient was in a good clinical condition, but EGD showed multiple gastric and duodenal small ulcers. Dose of azathioprine was optimized because patient refused biologics. After six months of asymptomatic period EGD showed mucosal healing. Azatioprine was continued. One year after the diagnosis of CD he was admitted again to our hospital because of intermittent fevers, severe periumbilical pain, weight loss and dehydration. Laboratory tests showed anemia (Hb 111 g/L), leucocytosis (L12.6/L) an elevated CRP (168 mg/L). CT scan detected jejunal perforation with multiple liver and spleen abscesses. He went under surgery with jejunal resection and histological findings from resected jejunum suggested EATL. In two postoperative days, the patient had progressive liver injury and suppression of bone marrow activity, and despite all supportive treatments he died.
Conclusion
EATL is a serious complication of coeliac disease, it rarely develops in young people and presents diagnostic challenge especially in CD patients. The aim of this report was to raise awareness of the importance of endoscopy with tissue sampling and cross sectional imaging especially in refractory coeliac disease and CD patients.
Studies on the nature and significance of connective tissue antibodies in adult coeliac disease and Crohn's disease