Late Presenting Celiac Disease and Atypical T-Cell Lymphoproliferative Disorder

Abstract

Celiac Disease (CeD) is often overlooked in the elderly. Delayed recognition can result in higher morbidity and mortality as it is associated with a risk of other autoimmune pathologies, malnutrition, and enteropathy-associated T cell lymphoma. Here, we present a patient diagnosed with late-onset CeD who was ultimately determined to have an atypical T-cell lymphoproliferative disorder. An 87-year-old female was admitted for evaluation of chronic watery diarrhea in the setting of recurrent readmissions for dehydration over the past several months. Symptoms remained unchanged despite several courses of vancomycin for C. difficile infection (based on PCR positive stool assay). She endorsed 15-lb weight loss. Medical history was notable for stage IV angioimmunoblastic T-cell lymphoma (ATCL), previously treated with chemotherapy and maintenance lenalidomide. Exam was notable for BMI of 19 and cachexia with temporal wasting; abdomen was soft, minimally distended, non-tender with no mass. Laboratory studies were notable for anemia (hemoglobin 9.1 g/dL), thrombocytopenia (102,000 platelets/μL), hypoalbuminemia (2.1 g/dL), and hypocalcemia (6.9 mg/dL). Stool tests for culture, Wright stain, alpha-1-antitrypsin, pancreatic elastase, calprotectin, chymotrypsin, and fecal fat were unrevealing. Vasoactive intestinal polypeptide, gastrin, and 5-hydroxyindoleacetic acid (5-HIAA) urine tests also were normal. Celiac serologies returned positive for tissue transglutaminase (72.2) and endomysial antibodies (1:80). CT scan demonstrated multiple prominent mesenteric lymph nodes throughout the GI tract. Upper endoscopy with duodenal biopsies confirmed diagnosis of CeD and duodenitis. Given her history of ATCL, molecular testing was performed, revealing clonal rearrangement distinct from her ATCL diagnosis. Overall, the features were consistent with an atypical T-cell lymphoproliferative disorder. She continued to have severe diarrhea despite strict adherence to a gluten free diet and was discharged to a skilled nursing facility where she died several weeks later. This case highlights the need for consideration of CeD when evaluating elderly patients presenting with diarrhea-especially since the risk of developing intestinal lymphoma in this population increases with advanced age. Our patient was ultimately diagnosed with a concurrent lymphoproliferative disorder involving the GI tract that was likely related to underlying CeD.