Celiac Disease, Enteropathy-Associated T-Cell Lymphoma, and Primary Sclerosing Cholangitis in One Patient: A Very Rare Association and Review of the Literature

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a very rare peripheral T-cell lymphoma which is mostly associated with celiac disease. However, the association of primary sclerosing cholangitis and enteropathy-associated T-cell lymphoma is uncommon. Herein we report and discuss the first case of patient who presented simultaneously with these two rare diseases. It is a 54-year-old man who stopped gluten-free diet after 15 years history of celiac disease. The diagnosis was based on the histological examination of duodenal biopsy and the diagnosis of primary sclerosing cholangitis was made on liver biopsy, as well as the magnetic resonance cholangiogram. The treatment of EATL is mainly based on chemotherapy in addition to the optimal management of complications and adverse events that impact on the response to treatment and clinical outcomes, although the prognosis remains remarkably very poor.

Review of Three Pediatric Wheat-Related Disorder Cases with Disparate Clinical Manifestations

“…Gradual autoimmune damage of the biliary tree leads to irreversible damage to bile ducts, persistent jaundice, ascites, varices, cholestasis, cirrhosis, liver failure, and death or liver transplantation within 10-20 years [56,73,77]. It is normally associated with inflammatory bowel disease and sometimes associated with celiac disease, chronic pancreatitis, diabetes mellitus, rheumatoid arthritis, or sarcoidosis [56,57]. Treatments are usually cholestyramine, ursodeoxycholic acid, fat-soluble vitamins, antibiotics for associated infection, and immune suppressant steroids.…”

“…Treatments are usually cholestyramine, ursodeoxycholic acid, fat-soluble vitamins, antibiotics for associated infection, and immune suppressant steroids. Surgical procedures are used to open up strictures of bile ducts in the liver and liver transplant is used for patients with end-stage liver disease [57]. There are no known treatments that bring about full remission from PSC [73,77].…”

The first three years of Autopsy and Case Reports: an interesting journey

“…During the past two years, the first citations began to emerge 27 - 31. If we exclude self-citations and consider only citations in indexed journals, the citation per article index in two years (calculated just as the Impact Factor) for 2013 (concerning the 2011-2012 biennium) is 0.0469.…”