Current Status of Cutaneous T-Cell Lymphoma: Molecular Diagnosis, Pathogenesis, Therapy and Future Directions

Primary cutaneous T-cell lymphomas (CTCL) represent a heterogeneous group of neoplasms characterized by skin-homing malignant T-lymphocytes. In contrast to primary extracutaneous (nodal) lymphomas, CTCL is characterized by a prolonged clinical course with a different clinical behavior and outcome. Disease progression, however, involves the recirculation compartments, i.e. lymph nodes and peripheral blood, and may finally spread to the visceral organs. Advances in T-cell receptor gene rearrangement techniques support the clinical diagnosis in early stages of CTCL by improved sensitivity and specificity of molecular diagnosis. The pathogenesis of CTCL is characterized by an altered immune biology and the accumulation of genetic mutations during disease progression. Although there is an initial response to standard therapy, including photochemotherapy, interferons, and retinoids, all patients will eventually relapse, and therefore treatment of CTCL continues to be palliative. New therapeutic drugs including bexarotene, DAB(389)IL-2 and IL-12 have demonstrated clinical responses; and new experimental therapeutic directions, e.g. stem cell transplantation and vaccination strategies may be applied with the intention to cure.