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Prepublished online as a Blood First Edition Paper on April 17, 2002; DOI 10.1182/blood-2001-12-0199.
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Blood, 15 July 2002, Vol. 100, No. 2, pp. 578-584
NEOPLASIA
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
From the Department of Dermatology,
Ludwig-Maximillians University Munich, Germany; and Department
of Dermatology, Institute of Pathology, University Medical Center
Benjamin Franklin, The Free University of Berlin, Germany.
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
isdespite the sometimes extremely long course of the diseaseno
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- 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.
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