Kinross-Wright John Jacob, Potu Kalyan Chakravarthy, Pownell Brandy, Lamfers Randall, Bleeker Jonathan S
Department of Internal Medicine, Sanford USD School of Medicine, Sioux Falls, SD, USA.
Department of Pathology, Sanford USD School of Medicine, Sioux Falls, SD, USA.
Case Rep Hematol. 2018 Mar 5;2018:6063519. doi: 10.1155/2018/6063519. eCollection 2018.
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.
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.
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.
爱泼斯坦-巴尔病毒（EBV）相关的淋巴增殖性疾病（LPD）是一种罕见疾病，通常发生在免疫功能低下的患者中。我们报告一例患有严重乳糜泻的患者发生EBV相关LPD的病例，这是首例描述该综合征在这种诊断患者中的报告。
一名69岁的白种女性，近期诊断为乳糜泻，尽管在就诊前数周坚持无麸质饮食，但仍因持续腹泻、腹痛、体重减轻和疲劳前来我院就诊。她接受了隐匿性恶性肿瘤评估，CT扫描发现腹腔内弥漫性肠系膜淋巴结肿大。肠系膜淋巴结活检显示EBV阳性、CD20阳性的混合性淋巴增殖过程，以T细胞为主，但无单克隆细胞群，认为符合EBV相关LPD。骨髓活检显示噬血细胞性淋巴组织细胞增生症，使她的病情复杂化。她接受了类固醇和利妥昔单抗治疗，但病情仍持续恶化，最终发展为耐甲氧西林金黄色葡萄球菌菌血症并死于该病。
据我们所知，这是乳糜泻、EBV相关LPD和噬血细胞性淋巴组织细胞增生症这一组合的首例报告。照顾患有严重、未控制的乳糜泻和腺病患者的医疗人员应考虑EBV相关LPD。
