Cutaneous T-cell lymphoma: 2011 update on diagnosis, risk-stratification, and management

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).

The diagnosis of MF or SS requires the integration of clinical and histopathologic data.

Tumor, node, metastasis, and blood (TNMB) 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, denileukin diftitox, and histone deacetylase inhibitors before escalating therapy to include systemic, single-agent chemotherapy. Multiagent chemotherapy may be used for those patients with extensive visceral involvement requiring rapid disease control. In highly-selected patients with disease refractory to standard treatments, allogeneic stem-cell transplantation may be considered.