Chronic active Epstein-Barr virus disease in a case of persistent polyclonal B-cell lymphocytosis

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.

Mitterer M, Pescosta N, Fend F, Larcher C, Prang N, Schwarzmann F,
Coser P, Huemer HP

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