A Rare Case of T-Cell Chronic Active Ebstein-Barr Virus (Caebv) Complicated by Development of Hemophagocytic Lymphohistiocytosis (Hlh), T Cell Lymphoproliferative Disorder (Lpd) and Ebv Meningitis

ABSTRACT

Background: T-cell chronic active Epstein-Barr virus infection (CAEBV) is a rare disease in which patients have the Epstein-Barr virus (EBV) present mainly in the T-cells, which infiltrate tissues like the liver, and bone marrow. Patients eventually develop liver failure, hemophagocytic lymph histiocytosis (HLH), coronary artery aneurysms, EBV infiltrating T-cells impairing organ function, or T-cell lymphomas. Prognosis is poor. The current treatment of choice is an allogeneic hematopoietic stem cell transplant. A study by the NIH and Baylor College of Medicine, which reviewed 28 years of data, only found 19 cases of CAEBV. We aim to report a rare case of T-cell chronic active Ebstein-Barr Virus (CAEBV) complicated by the development of HLH and T-Cell LPD. Method(s) A chart review of the CAEBV patient was performed, focusing on disease progression, treatment plans, and complications. Result(s) A 45-year- old Latin American woman from Mexico initially presented with abnormal liver enzymes after taking herbal medications. The patient underwent a liver biopsy and was initiated on prednisone for possible autoimmune hepatitis pending the biopsy report. The liver biopsy showed EBV hepatitis with EBV positive atypical T-cell infiltrate with steatohepatitis and marked steatosis. Prednisone was stopped, and the patient was referred to Hematology. Plasma EBV level was elevated to 3300 IU/mL. The patient was readmitted for sepsis and pancytopenia prior to being seen by Hematology. Bone marrow biopsy showed EBV+T-cell LPD and HLH, and the patient was started on dexamethasone and rituximab. The patient improved, and dexamethasone was weaned off. Subsequently the patient has had numerous hospital admissions for ESBL UTI, CoNS bacteremia, aspiration pneumonia, vocal cord dysfunction, EBV pneumonia, PCP pneumonia, chemotherapy-induced neuropathy, neutropenic fever, chronic respiratory failure and EBV reactivation. The patient underwent multiple rounds of chemotherapy with rituximab and R-CHOP regimen for persistent HLH. In spite of the treatment, the patient developed EBV encephalitis, further complicated by COVID -19 infection. Her family opted for comfort care, and the patient passed away in the hospital. Conclusion(s) Approximately 95% of adults are infected with EBV at some point in their lives and are asymptomatic in most cases. Very rarely do patients develop CAEBV -a life-threatening disease. Allogeneic stem cell transplant should be considered early on in the disease. Unfortunately, our patient had social factors such as lack of insurance and social support that prevented her from getting a timely stem cell transplant. (Figure Presented).