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Chronic active Epstein‐Barr virus disease in a case of persistent polyclonal B‐cell lymphocytosis
Author(s) -
MITTERER MANFRED,
PESCOSTA NORBERT,
FEND FALKO,
LARCHER CLARA,
PRANG NADJA,
SCHWARZMANN FRITZ,
COSER PAOLO,
HUEMER HARTWIG P.
Publication year - 1995
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1995.tb05579.x
Subject(s) - biology , virology , immunology , virus , epstein–barr virus , bzlf1 , lymphoproliferative disorders , herpesviridae , viral disease , lymphoma
Summary. Persistent polyclonal B‐cell lymphocytosis (PPBL) is a rare haematological disorder. It is characterized by activated and morphologically atypical B lymphocytes and polyclonal IgM production and has been associated with female sex, cigarette smoking, and HLA‐DR7 expression. We report a case of PPBL with intermitting symptoms compatible with a chronic fatigue syndrome, recurrent erythema nodosum and multiforme. Serological findings suggested a chronic active Epstein‐Barr virus (EBV) infection. Messenger RNA of EBV immediate early gene transactivation BZLF1 was detected in peripheral blood lymphocytes by reverse transcriptase PCR indicating a persistent replication of the virus. Over 2 years of observation we detected varying numbers of atypical lymphocytes. These cells hybridized with a probe specific for the EBV internal repeat region (BamHI W) which indicates a productive infection. Of interest, no reaction was observed with a probe specific for the latency‐associated small RNAs (EBERs). The immunological phenotype of the polyclonal B cells was similar to B‐cell lines immortalized by EBV in vitro , expressing a number of activation molecules (CD23, CD25, CD54) and the bcl‐2 protein. In summary, our findings suggest that persistent EBV replication might be crucial in the development of lymphoproliferative disorders such as PPBL.