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Cutaneous T-cell lymphomas (CTCLs) are subdivided by lesion morphology, behavior, and surface receptors. Mycosis fungoides (MF) and Sézary syndrome (SS) are derived from CD4+ effector or central memory T-cells respectively. MF presents clinically as patches, plaques, or tumors, and SS presents with erythroderma. After MF/SS, the next most common CTCLs are CD30+ lymphoproliferative disorders: self-regressing lymphomatoid papulosis (LyP) or tumors of anaplastic large-cell lymphoma (ALCL), which express high levels of tumor necrosis factor death receptor member 8, also called CD30. Although MF is not considered to be a CD30+ lymphoproliferative disorder, MF may co-exist with LyP lesions, and MF may express CD30, especially in the setting of large-cell transformation. The development of targeted therapy for CD30+ CTCLs will help in understanding the importance of the CD30 death receptor in pathogenesis and will improve treatment options.
Current Hematologic Malignancy Reports – Springer Journals
Published: Aug 18, 2011
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