TY - JOUR
T1 - Primary cutaneous T-cell lymphoma expressing FOXP3
T2 - A case report supporting the existence of malignancies of regulatory T cells
AU - Marzano, Angelo V.
AU - Vezzoli, Pamela
AU - Fanoni, Daniele
AU - Venegoni, Luigia
AU - Berti, Emilio
PY - 2009/8
Y1 - 2009/8
N2 - Regulatory T (Treg) cells, which represent 5% to 10% of peripheral T cells, regulate the activities of T-cell subsets by performing immunosuppressive functions and thus preventing the development of autoimmune responses. The majority of Treg cells are CD4+, CD25+, and FOXP3+. Recently, it has been demonstrated that the tumor cells in adult T-cell leukemia lymphomas can function as Treg, raising the question of whether any variant of primary cutaneous T-cell lymphoma may also express a regulatory phenotype. We describe an extraordinary case of primary cutaneous T-cell lymphoma clinically characterized by protean cutaneous manifestations and histologically showing a pattern consistent with epidermotropic pleomorphic medium-/large-cell primary cutaneous T-cell lymphoma. The majority of neoplastic cells were CD4+ CD25+ T cells and strongly expressed FOXP3. With this background, the current case, characterized by an aggressive course requiring polychemotherapy, may support the existence of lymphoproliferative malignancies of Treg cells.
AB - Regulatory T (Treg) cells, which represent 5% to 10% of peripheral T cells, regulate the activities of T-cell subsets by performing immunosuppressive functions and thus preventing the development of autoimmune responses. The majority of Treg cells are CD4+, CD25+, and FOXP3+. Recently, it has been demonstrated that the tumor cells in adult T-cell leukemia lymphomas can function as Treg, raising the question of whether any variant of primary cutaneous T-cell lymphoma may also express a regulatory phenotype. We describe an extraordinary case of primary cutaneous T-cell lymphoma clinically characterized by protean cutaneous manifestations and histologically showing a pattern consistent with epidermotropic pleomorphic medium-/large-cell primary cutaneous T-cell lymphoma. The majority of neoplastic cells were CD4+ CD25+ T cells and strongly expressed FOXP3. With this background, the current case, characterized by an aggressive course requiring polychemotherapy, may support the existence of lymphoproliferative malignancies of Treg cells.
UR - http://www.scopus.com/inward/record.url?scp=67650473615&partnerID=8YFLogxK
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U2 - 10.1016/j.jaad.2008.11.894
DO - 10.1016/j.jaad.2008.11.894
M3 - Article
C2 - 19615546
AN - SCOPUS:67650473615
VL - 61
SP - 348
EP - 355
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 2
ER -