title: Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin.
abstract: Lymphomatoid papulosis (LyP) represents an intriguing cutaneous T-cell lymphoproliferative disorder with a histologic appearance resembling malignant lymphoma. This finding strongly contrasts with the benign clinical course of the disease. However, in 10% to 20% of cases, LyP can precede, coexist with, or follow malignant lymphoma. In these cases, the same T-cell population has been shown to be present in the LyP as well as in the associated lymphoma. In most LyP cases, there is-despite the sometimes extremely long course of the disease-no evolution of a secondary lymphoma. The investigation of these uncomplicated LyP cases for the presence of clonal T-cell receptor rearrangements has produced heterogeneous results. This might be explained by biologic or technical reasons arising from analyzing whole tissue DNA extracts. To definitively clarify whether the large atypical CD30(+) cells in LyP without associated lymphoma all belong to the same clone or represent individually rearranged T cells, we analyzed the T-cell receptor-gamma rearrangements of single CD30+ as well as of single CD30- cells isolated from 14 LyP lesions of 11 patients. By using this approach we could demonstrate that the CD30+ cells represent members of a single T-cell clone in all LyP cases. Moreover, in 3 patients the same CD30+ cell clone was found in anatomically and temporally separate lesions. In contrast, with only a few exceptions, the CD30- cells were polyclonal in all instances and unrelated to the CD30+ cell clone. Our results demonstrate that LyP unequivocally represents a monoclonal T-cell disorder of CD30+ cells in all instances.
authors.affiliation: Department of Dermatology, Ludwig-Maximillians-University Munich, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Steinhoff
authors.forename: Michael
authors.initials: M
authors.lastname: Hummel
authors.forename: Ioannis
authors.initials: I
authors.lastname: Anagnostopoulos
authors.forename: Peter
authors.initials: P
authors.lastname: Kaudewitz
authors.forename: Volkhard
authors.initials: V
authors.lastname: Seitz
authors.forename: Chalid
authors.initials: C
authors.lastname: Assaf
authors.forename: Christian
authors.initials: C
authors.lastname: Sander
authors.forename: Harald
authors.initials: H
authors.lastname: Stein
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002999:Clone Cells / Q000276:immunology / Q000473:pathology*; D005260:Female; D015321:Gene Rearrangement; D019675:Genes, T-Cell Receptor gamma / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017730:Ki-1 Antigen / Q000032:analysis*; D017731:Lymphomatoid Papulosis / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D017422:Sequence Analysis, DNA; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*
chemical_list: D017730:Ki-1 Antigen