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Prepublished online as a Blood First Edition Paper on April 17, 2002; DOI 10.1182/blood-2001-12-0199.
Submitted December 11, 2001
Accepted March 3, 2002
Single Cell Analysis of CD30+ Cells in Lymphomatoid Papulosis demonstrates a Common Clonal T-Cell Origin
Matthias Steinhoff, Michael Hummel*, Ioannis Anagnostopoulos, Peter Kaudewitz, Volkhard Seitz, Chalid Assaf, Christian Sander, and Harald Stein
Department of Dermatology, Klinikum Benjamin Franklin, Berlin, Germany
Institute of Pathology, Klinikum Benjamin Franklin, Berlin, Germany
Department of Dermatology, Ludwig-Maximillians-University, Munich, Germany
* Corresponding author; email: hummel{at}ukbf.fu-berlin.de.
Lymphomatoid papulosis (LyP) represents an intriguing cutaneous T-cell lymphoproliferative disorder with a histological appearance resembling malignant lymphoma. This finding strongly contrasts with the benign clinical course of the disease. However, in 10 to 20 percent of cases, LyP can precede, co-exist 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 the majority of 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 (TCR) rearrangements has produced heterogeneous results. This might be explained by biological 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 TCR-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 three 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.
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