Blood, Vol. 94 No. 9 (November 1), 1999:
pp. 3077-3083
CD30-CD30 Ligand Interaction in Primary Cutaneous CD30+
T-Cell Lymphomas: A Clue to the Pathophysiology of Clinical Regression
M. Mori,
C. Manuelli,
N. Pimpinelli,
C. Mavilia,
E. Maggi,
M. Santucci,
B. Bianchi,
P. Cappugi,
B. Giannotti, and
M.E. Kadin
From the Departments of Dermatology, Internal Medicine
(Immunoallergology and Respiratory Disease Unit), and Human Pathology
and Oncology, University of Florence Medical School, Florence, Italy;
and the Department of Pathology-Section of Hematopathology, Beth Israel
Deaconess Medical Center, Harvard Medical School, Boston, MA.
Primary CD30+ cutaneous T-cell lymphomas (CTCLs)
represent a spectrum of non-Hodgkin's lymphomas (NHLs) that have been
well defined at the clinical, histologic, and immunologic level. This group, which includes 2 main entities (large cell lymphoma and lymphomatoid papulosis [LyP]) and borderline cases, is characterized by the expression of CD30 antigen by neoplastic large cells at presentation, possible spontaneous regression of the skin lesions, and
generally favorable clinical course. Although the functional relevance
of CD30 and its natural ligand (CD30L) expression in most cases of NHL
is presently undefined, previous studies indicate that CD30L is likely
to mediate reduction of proliferation in CD30+ anaplastic
large-cell NHL. No information is currently available concerning the
expression of CD30L in primary CD30+ CTCLs. In this
study, we investigated the immunophenotypic and genotypic expression of
CD30 and CD30L in different developmental phases of skin lesions
(growing v spontaneously regressing). By immunohistochemistry,
CD30L expression was detected in regressing lesions only; by molecular
analysis, the expression of CD30L was clearly higher in regressing
lesions than in growing ones. CD30L, while expressed by some small
lymphocytes, was most often coexpressed by CD30+
neoplastic large cells, as demonstrated by 2-color immunofluorescence and by immunohistochemistry on paraffin sections. Taken together, these
data suggest that CD30-CD30L interaction may play a role in the
pathobiology of primary cutaneous CD30+
lymphoproliferative disorders. In particular, CD30L (over)expression might have a major role in the mechanism of self-regression of skin
lesions, the most distinctive clinical feature of this cutaneous lymphoma subtype.
