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
M
Department of Haematology, General Hospital, Bozen, Italy.
Pescosta
N
N
Fend
F
F
Larcher
C
C
Prang
N
N
Schwarzmann
F
F
Coser
P
P
Huemer
H P
HP
D015703:Antigens, CD / Q000032:analysis; D001402:B-Lymphocytes / Q000821:virology*; D001483:Base Sequence; D002908:Chronic Disease; D015673:Fatigue Syndrome, Chronic / Q000150:complications; D005260:Female; D015809:HLA-DR7 Antigen / Q000032:analysis; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D008218:Lymphocytosis / Q000821:virology*; D008875:Middle Aged; D008969:Molecular Sequence Data; D012333:RNA, Messenger / Q000032:analysis; D014412:Tumor Virus Infections / Q000150:complications*
D015703:Antigens, CD; D015809:HLA-DR7 Antigen; D012333:RNA, Messenger