Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder Presenting as Chronic Diarrhea and Intestinal Bleeding: A Case Report

Abstract

Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease (EBV+ T-LPD) is extremely rare. Primary acute or chronic active Epstein-Barr virus infection triggers EBV+ T-LPD's onset and the disease involves clonal proliferation of infected T-cells with activated cytotoxic phenotype. The adult-onset EBV+ T-LPD (ASEBV+ T-LPD) is even rarer and needs to be extensively studied. Further, according to literature review, it is a challenge to find patients who are immunocompetent and diagnosed with ASEBV+ T-LPD involving gastrointestinal tract. This case report discusses a previously healthy middle aged woman who presented with unique symptoms mimicking inflammatory bowel disease, and required a total colectomy and terminal ileum rectomy, as reveled by endoscopic examinations, due to severe gastrointestinal bleeding. Post-surgery histopathological findings were confirmatory for the diagnosis of ASEBV+ T-LPD (II: Borderline). This patient died 7 months after the diagnosis.

Indolent T-Cell Lymphoproliferative Disease of the GI Tract: Insights for Better Diagnosis, Prognosis, and Appropriate Therapy

“…Indolent GI T-LPD cases may rarely be associated with EBV infections. Wang et al has recently reported a very rare case of adult-onset, immunocompetent EBV + GI T-LPD (8). This case was a middle-aged woman with chronic diarrhea and severe intestinal bleeding.…”

Gastric ulcer as the main complication in hemophagocytic lymphohistiocytosis: A case report

“…EBV infects about 95% of human beings worldwide, although most of them are asymptomatic. 5 However, it is involved in the pathogenesis of lymphoma and HLH. The latter is a rare but life-threatening disease characterized by uncontrolled hyperinflammation and subsequent systemic organ failure.…”

“…3 While gastrointestinal involvement is relatively uncommon (18%), and mostly occurs in the intestine. 5 patients with permanent remission, but it can be done only when the patient's condition is stable. 5,8 In conclusion, we described here a patient initially diagnosed as gastric ulcers, but finally identified as having HLH with EBV infection.…”

A new therapy in Epstein-Barr virus-associated lymphoproliferative disease: a case report and a revision of the literature

“…Chronic active Epstein-Barr virus infection (CAEBV) is a systemic EBV-positive lymphoproliferative disorder (EBV-LPD), which is marked by persistent infectious mononucleosis-like symptoms. CAEBV may result in life-threatening complications [1–6] such as malignant lymphomashepatic failure, interstitial pneumonia, coronary artery aneurysms, central nervous system (CNS) involvement, hemophagocytic syndromes and massive hemorrhage from the gastrointestinal tract. However, it is very rare for a patient to have several synchronous symptoms owing to EBV infection.…”