An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare disease found in the small bowel and is seen most commonly in patients with refractory celiac disease (RCD). We present a case of an elderly male with celiac disease (CD) diagnosed in childhood with months of abdominal pain and diarrhea despite attempting to avoid gluten in his diet. After persistent symptoms for months, the patient was admitted for an acute abdomen and was found to have small bowel perforation due to a jejunal mass that was diagnosed as an EATL. In 2–5% of adult onset CD, serious complications such as RCD or malignancy develop. The clinical course for EATL is aggressive and generally has a poor prognosis. This case highlights the importance of early clinical suspicion for a small bowel malignancy in patients with a long-standing history of CD and acute worsening of symptoms. Early workup and diagnosis is vital in improving morbidity and mortality in patients with EATL.

Peripheral T-Cell Lymphoma Possibly Due to Unrecognized Celiac Disease in an Elderly Patient: A Case Report

“…Intestinal lymphomas are believed to be one of the leading causes of death in patients with CD diagnosed in adulthood [ 3 ]. The incidence of lymphoma is much greater in the sixth, seventh, and eighth decades of life.…”

“…Lymphomas generally follow a highly aggressive clinical course and have a poor prognosis [ 9 ]. EATLs can also present in disseminated forms with the involvement of mesenteric lymph nodes, the liver, spleen, lungs, and skin [ 3 ]. EATLs should be suspected in patients with elevated LDH and β2microglobulin, anemia, a BMI of around 18, and low serum albumin levels [ 12 ].…”

“…It has been shown that 2–3% of patients with CD will develop intestinal lymphoma, of which 65% is of T-cell origin. These lymphomas are believed to be one of the leading causes of death in patients with CD diagnosed in adulthood [ 3 ]. The risk of malignancies increases in older celiac patients and is associated with both the duration of gluten exposure and age.…”

A fatal outcome in a patient with coeliac disease who suspended a strict gluten-free diet: a case report

“…1 It has been reported that approximately 70% of patients with CD have symptomatic remission within 2 weeks after starting a GFD. 2 Herein, this case report presents a patient with recurrent CD due to a discontinuation of GFD. When the CD recurred, the patient developed fever and pancytopaenia, and quickly died of haemophagocytic lymphohistiocytosis (HLH).…”