[ XXXX of the S  zary and Bouvrain type. Progress in physiopathologic and therapeutic outlook].

@entity1 @entity404 @entity55965 @entity706 @entity5 @entity784 @entity6977 @entity1199 @entity405
In 1938, S  zary and Bouvrain reported a new form of disseminated @entity706 , characterized by an erythrodermia, and by the presence in the blood of cells with a cerebriform nucleus. It was shown later that this disease was related to the proliferation of a subpopulation of @entity404 + T lymphocytes. With the aim to develop new therapeutic strategies for these @entity706 , we tried to evidence and to characterize the @entity5 -specific immune responses in these @entity784 . We have isolated several T cell clones from lymphocytes infiltrating a @entity1 MHC class II negative @entity784 . We describe here two of these clones, TC5 and TC7, with respectively a @entity404 + @entity405 + and @entity404 + @entity405 - phenotype. Both clones mediated a specific MHC class I-restricted cytotoxic activity toward the fresh @entity5 cells, and @entity5 cell lines previously established with @entity1199 and @entity6977 from the skin and from the blood. Analysis of the T-cell receptor V beta gene expression revealed that the @entity5 cells, that were shown to have a trisomy 7 by fluorescent in situ hybridization, expressed V beta 7/J beta 2.3, V beta 13/J beta 2.5 and V beta 22/J beta 2.5 rearrangements. Phenotypic analysis using specific anti-V beta monoclonal antibodies indicated that only V beta 13 could be detected on the cell membrane of the @entity5 cells. Analysis of the TCR V beta gene expression of the clones showed that TC5 and TC7 expressed a unique TCR-V beta transcript, corresponding respectively to V beta 5/J beta 2.3 and V beta 17/J beta 2.7 gene segments. To determine whether these reactive T lymphocytes were present in vivo, we used specific primers corresponding to TC5 and TC7-V beta TCR transcripts. The results demonstrated that both cytotoxic T cell clones were present at the lesional skin site and amplified in vitro. @entity55965 was found in the @entity1 peripheral blood invaded by tumoral cells, whereas TC5 was not, indicating that the repertoire of the reactional lymphocytes differs in the blood and at the @entity5 site. These results demonstrate for the first time the presence of reactive T lymphocytes with @entity404 or double positive phenotype infiltrating a CTCL. These findings raise new perspectives for the treatment of @entity784 .
