Hemophagocytic Lymphohistiocytosis in Refractory Celiac Disease Type II Transformed into Enteropathy-Associated T-Cell Lymphoma

Abstract

Celiac disease (CD) can lead to small intestinal damage from gluten exposure. Refractory celiac disease type II (RCD2), a complication of CD, can transform to enteropathy-associated T-cell lymphoma (EATL). Hemophagocytic lymphohistiocytosis (HLH) is a rare complication that has been reported in patients with CD, RCD2, and EATL. We report the case of a 61-year-old man with a history of CD complicated by RCD2 that transformed into EATL with HLH as its presenting syndrome.