Prognostic Factors for Chronic Active Epstein‐Barr Virus Infection

Abstract

Chronic active Epstein-Barr virus infection (CAEBV) is a high-mortality and high-morbidity disease. To clarify the prognostic factors, a national survey was performed in Japan, and data for 82 patients who met the criteria for CAEBV were analyzed. Of these 82 patients, 47 were alive and 35 had already died. Multivariate analysis revealed that thromobocytopenia and age at disease onset were correlated with mortality. The probability of 5-year survival was 0.45 for older patients (onset age, > or = 8 years), 0.94 for younger patients (P<.001), 0.38 for patients with thrombocytopenia (platelet count < 12 x 10(4) platelets/microL at diagnosis), and 0.76 for patients without thrombocytopenia (P=.01). Furthermore, patients with T cell infection by EBV had shorter survival times than patients with natural killer cell infection (probability of 5-year survival, 0.59 vs. 0.87; P<.009). Patients with CAEBV with late onset of disease, thrombocytopenia, and T cell infection had significantly poorer outcomes.

Measuring Epstein–Barr virus (EBV) load: the significance and application for each EBV‐associated disease

“…There is accumulating evidence that the clonal expansion of EBV-infected T or NK cells plays a central role in the pathogenesis of chronic active EBV infection [93][94][95]. We proposed that this disease consists of a T cell-or NK cell-type disease, based on the results of PBMC fractionation and subsequent quantitative PCR [96][97][98]. EBVinfected T or NK cells with a latency type II pattern can evade the host cellular immune system due to the limited expression of viral proteins with reduced antigenicity [27,98,99].…”

Proposed guidelines for diagnosing chronic active Epstein‐Barr virus infection

“…The following clinical features are commonly noted in patients with CAEBV [19][20][21]. Symptoms are persistent and chronic and generally consist of prolonged or intermittent fever, lymphadenopathy, and/or hepatosplenomegaly.…”

“…We recently demonstrated in a national Japanese survey that patients with CAEBV had poorer outcomes when associated with late onset of disease, thrombocytopenia, and EBV infection of T cells [20].…”

EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features

“…Likewise, case 4 fell into category A2 for polymorphic infiltration with clonal rearrangements of TCR gene and case 3 was classified into category A3 for monomorphic infiltration with clonal proliferation. Category A1 and A2 corresponding to smoldering state and chronic state, respectively [8] whose 5 years overall survival (OS) rate is 70% showed better prognosis than category A3 whose 5 years OS rate is less than 30% [16]. However, our two patients of category A1 who refused to receive further treatment and died within one year cannot objectively reflect the process of disease.…”