EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease

• Published in: Case reports in hematology Vol. 2018; no. 2018; pp. 1 - 4
• Main Authors: Lamfers, Randall, Pownell, Brandy, Chakravarthy Potu, Kalyan, Kinross-Wright, John Jacob, Bleeker, Jonathan S.
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Publishing Corporation 01-01-2018; Hindawi; John Wiley & Sons, Inc; Hindawi Limited
• Subjects: Abdomen; B cells; Case Report; Case reports; Celiac disease; Diagnostic imaging; Diarrhea; Epstein-Barr virus; Family medical history; Gastroenterology; Gluten; Hematology; Hepatology; HIV; Human immunodeficiency virus; Lymphatic diseases; Lymphoma; Methicillin; Monoclonal antibodies; Older people; Patients; Population; Small intestine; T cells; Targeted cancer therapy; Transplants & implants; Viruses; Weight loss
• ISSN: 2090-6560; 2090-6579
• DOI: 10.1155/2018/6063519

Background. Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. Case Summary. A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation. She underwent evaluation for occult malignancy and was found to have diffuse intra-abdominal mesenteric lymphadenopathy on CT scan. Biopsy of mesenteric nodes revealed an EBV positive, CD20 positive mixed lymphoproliferative process with T-cell predominance, but without a monoclonal cell population felt to be consistent with EBV-associated LPD. Bone marrow biopsy revealed hemophagocytic lymphohistiocytosis, complicating her course. She was treated with steroids and rituximab but continued to decline, eventually developing MSSA bacteremia and succumbing to her disease. Conclusion. To our knowledge, this is the first report of the constellation of celiac sprue, EBV-associated LPD, and hemophagocytic lymphohistiocytosis. Providers caring for patients with severe, uncontrolled celiac disease and adenopathy should consider EBV-associated LPD.