The clinical characteristics and the features of immunophenotype of peripheral lymphocytes of adult onset chronic active Epstein-Barr virus disease at a Tertiary Care Hospital in Beijing

Abstract

Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected.There were 28 adult onset CAEBV patients. The median age was 45 (range, 20–81). Most of the patients presented with fever; splenomegaly; lymphadenopathy and hepatitis. Unlike pediatric cases reported, the manifestations of cardiovascular diseases in our patients were pulmonary arterial hypertension, decreased cardiac function and aorta vasculitis. Prevalence of interstitial pneumonitis in our patients were comparatively higher and prevalence of hypersensitivity to mosquito bites were comparatively lower than that reported by Japan. In this study, CAEBV patients had decreased B cell, NK cell, CD4 cell and CD8 cell counts. The prevalence of low level of B cells, NK cells, CD4 cells was relatively higher than reported ever.Chinese adult onset CAEBV patients have different clinical characteristics and are featured by an immunosuppression status as demonstrated by decreased B cell, NK cell, CD4 cell and CD8 cell.

Chemotherapy Improved Pulmonary Arterial Hypertension in a Patient with Chronic-Active Epstein-Barr Virus Infection

“…Luo, et al studied the clinical characteristics and the features of adult CAEBV. 1) In 28 patients with CAEBV, 4 patients developed PAH. The clinical classification of pulmonary hypertension includes 5 groups according to their clinical presentation and pathological findings.…”

“…CAEBVassociated PAH is very rare, and is reported predominantly in East-Asian countries. [1][2][3] However, the treatment for CAEBV-associated PAH is not established. We report here a case of improved pulmonary hypertension after chemotherapy in a patient with CAEBV-associated PAH.…”

Epstein-Barr Virus-Positive T/NK-Cell Lymphoproliferative Diseases in Chinese Mainland

“…In China, there were only two retrospective studies on the clinical features of CAEBV systematically, one is about pediatric patients and the other is about adult cases ( 2 , 3 ). In total, eighty one CAEBV patients were reported, including 53 children with a mean age of 6.3 years (ranging from 6 months to 15 years) and 28 adults with a median age of 45 years (ranging from 20 to 81 years).…”

“…The number of lymphocytes, NK cell, B cell, total T cell, CD4 + T cell, and CD8 + T cell in CAEBV were lower than that in acute EBV infection ( 4 ). In adult onset CAEBV patients, the B cell, NK cell, CD4 + T cell and CD8 + T cell counts were also decreased ( 3 ).…”

Chronic active Epstein–Barr virus infection with intrapulmonary shunting: A case report

“…Liver failure is one of the major causes of mortality. Lung involvements of CAEBV such as pulmonary hypertension and interstitial pneumonitis are occasionally observed in cases of CAEBV [2], whereas intrapulmonary shunting in patients with CAEBV is rare. Herein, we reported a case of severe hypoxemia caused by intrapulmonary shunting related to CAEBV.…”