Somatic rearrangement of the TP63 gene preceding development of mycosis fungoides with aggressive clinical course

Cutaneous T-cell lymphomas (CTCLs) comprise a heterogeneous spectrum of T-cell neoplasms with widely varying clinical presentation, biologic behavior and overall outcome. 1 , 2 The most common CTCL is mycosis fungoides (MF), which can range from a localized, indolent process to an aggressive lymphoma with widespread cutaneous and extracutaneous involvement and large-cell transformation (MF-LCT). This biologic and clinical heterogeneity is a feature shared with other T-cell lymphomas, including systemic peripheral T-cell lymphomas (PTCLs). Also common to both CTCLs and PTCLs is a limited understanding of genetic mechanisms of pathogenesis and progression, the elucidation of which could facilitate classification, prognostication and individualized therapy. For example, accurate classification of CTCLs often requires careful clinical and pathological follow-up over time, and an enhanced understanding of CTCL genetics might allow earlier, more definitive classification. Furthermore, genetic biomarkers that identify patients at greatest risk of aggressive clinical behavior could allow initiation of earlier or more intensive treatment protocols that might lead to better outcomes. Finally, knowledge of CTCL genetics could improve biologic understanding of this group of diseases and facilitate the development of more specific, targeted therapies.