Cutaneous T‐cell lymphoma: 2014 Update on diagnosis, risk‐stratification, and management

Disease overview: Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data. Risk‐adapted therapy: TNMB (tumor, node, metastasis, and blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with significant nodal, visceral or blood involvement are generally approached with biologic‐response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single‐agent chemotherapy. Multiagent chemotherapy (e.g., CHOP) may be employed for those patients with extensive visceral involvement requiring rapid disease control. In highly selected patients, allogeneic stem‐cell transplantation may be considered. Am. J. Hematol. 89:837–851, 2014. © 2014 Wiley Periodicals, Inc.