title: HTLV-1-like particles and HTLV-1-related DNA sequences in an unambiguous case of Sèzary syndrome.
abstract: An unambiguous case of Sèzary syndrome associated with the presence of unusual retroviral infection markers is described. The blood smear showed 15% typical Sèzary cells but also rare atypical lymphocytes with convoluted nuclei, evocative of characteristic adult T-cell leukemia (ATL) flower cells. However, the patient did not present any clinical or biological manifestations of ATL, and human T-cell leukemia virus type 1 (HTLV-1) serology was consistently negative. After being cultured for 4 months, peripheral blood mononuclear cells (PBMC) produced typical type C retrovirus-like particles with budding forms strongly resembling HTLV-1 virions. The producer cells did not express HTLV-1-specific antigens detectable by indirect immunofluorescence (IIF). Southern blotting of uncultured PBMC DNA, submitted to digestion with the restriction enzymes PstI and SacI, and hybridized with a full genomic HTLV-1 probe, showed the presence of specific homologous sequences, absent in all of the healthy donor control PBMC DNAs. These HTLV-1-like sequences presented a restriction enzyme pattern distinct from that of the HTLV-1 prototype genome and of other HTLV-1 proviruses studied up to now. Polymerase chain reaction (PCR) with highly conserved HTLV-1 derived pol and env primers was consistently negative with the patient's DNA. All these results taken together suggest that our patient carries a retroviral agent partially homologous to, but probably different from HTLV-1. The possibility is discussed that this type of retroviral agent might be associated with a subtype of cutaneous T-cell lymphoma (CTCL) represented by a typical Sèzary syndrome with a very low percentage of ATL-like flower cells in the blood smear.
authors.affiliation: UPR A0043 CNRS Rétrovirus et Rétrotransposons des Vertébrés, Hôpital Saint-Louis, Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Bazarbachi
authors.forename: F
authors.initials: F
authors.lastname: Saal
authors.forename: L
authors.initials: L
authors.lastname: Laroche
authors.forename: J
authors.initials: J
authors.lastname: Lasneret
authors.forename: A
authors.initials: A
authors.lastname: Gessain
authors.forename: M T
authors.initials: MT
authors.lastname: Daniel
authors.forename: J
authors.initials: J
authors.lastname: Périès
mesh_terms: D000328:Adult; D015139:Blotting, Southern; D002478:Cells, Cultured; D004279:DNA, Viral / Q000097:blood*; D015490:HTLV-I Infections / Q000097:blood; D015368:Human T-lymphotropic virus 1 / Q000235:genetics*; D006801:Humans; D007963:Leukocytes, Mononuclear / Q000382:microbiology; D008297:Male; D008854:Microscopy, Electron; D016133:Polymerase Chain Reaction; D012751:Sezary Syndrome / Q000097:blood* / Q000235:genetics / Q000382:microbiology*; D014771:Virion / Q000235:genetics
chemical_list: D004279:DNA, Viral