Proposed guidelines for diagnosing chronic active Epstein‐Barr virus infection

Abstract

Since the initial report of unusual manifestations possibly associated with chronic active Epstein‐Barr virus (EBV) infection (CAEBV), nearly three decades have passed. During this period, reported cases with this entity have dramatically increased in the world. Additionally, recent development of diagnostic procedures, including molecular biological and immunological techniques, have provided us with the ability to define certain diseases, especially malignant disorders. Guidelines, derived mainly from the current literature and recent experiences with CAEBV in Japan, for diagnosing CAEBV are proposed to clarify this enigmatic disease. Am. J. Hematol. 80:64–69, 2005. © 2005 Wiley‐Liss, Inc.

Clonal expansion of Epstein–Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions

“…It is also known that patients with CAEBV may present with various cutaneous manifestations including HV-like eruptions [3]. CAEBV patients with HV-like eruptions sometimes develop cutaneous T-cell lymphoma that is defined as HV-like lymphoma in the 2008 World Health Organization classification of lymphoid neoplasms [19].…”

“…However, EBV infection has also been etiologically associated with various malignancies and lymphoproliferative disorders including chronic active EBV infection (CAEBV), in which monoclonal or oligoclonal proliferation of EBVinfected T or natural killer (NK) cells may play important roles in disease development [2]. CAEBV is characterized by persistent or recurrent infectious mononucleosis-like symptoms such as fever, lymphadenopathy and hepatosplenomegaly, and by extremely high viral loads in peripheral blood [3]. Patients with CAEBV may also suffer from cutaneous lesions including hypersensitivity to mosquito bites or hydroa vacciniforme (HV)-like eruptions.…”

Application of flow cytometric in situ hybridization assay to Epstein–Barr virus‐associated T/natural killer cell lymphoproliferative diseases

“…(10,(22)(23)(24) Diagnoses of HLH were made on the basis of criteria proposed by an international treatment study group, (25) whereas SCA-EBV was diagnosed using previously proposed criteria. (7,26) Briefly, for a diagnosis of SCAEBV to be made, patients had to fulfill the following diagnostic criteria: (i) an illness of >6 months duration (an EBV-related illness or symptoms including fever, persistent hepatitis, extensive lymphadenopathy, hepatosplenomegaly, pancytopenia, uveitis, interstitial pneumonia, hydroa vacciniforme, or hypersensitivity to mosquito bites); (ii) increased quantities of EBV in either affected tissues or peripheral blood; and (iii) no evidence of any prior immunologic abnormalities or of any other recent infection that may explain the condition. There were several overlapping cases.…”

Evaluation of the Epstein-Barr Virus R-Gene Quantification Kit in Whole Blood with Different Extraction Methods and PCR Platforms

“…2,4 More recently, EBV DNA load measurement in blood also appears to be a potentially helpful tool for monitoring other EBV-associated cancers such as nasopharyngeal carcinoma, AIDS-associated lymphoma, and Hodgkin's disease. [5][6][7][8] EBV DNA load measurements may also provide characterization of the natural history of IM. 9,10 However, an appropriate standardization of EBV DNA load measurement is still needed to accurately establish the predictive value of EBV DNA load in specific clinical situations.…”