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

Abstract

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: 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. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc.

Incidence and survival patterns of cutaneous T-cell lymphomas in the United States

“…Close to 50% of MF cases with known staging reported to SEER were stage 1A, disease with limited patches or plaques, and more than 85% were early stage disease (stage 1A, 1B, IIA). Early and advanced stage MF differ starkly in mortality, [36] and mirroring the increased HRs for advanced stage disease seen in our study. Staging of MF in SEER is according to the 1979 National Cancer Institute (NCI) and MF Cooperative Group TNM(B) staging classification system.…”

A single center phase II study of ixazomib in patients with relapsed or refractory cutaneous or peripheral T‐cell lymphomas

“…Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma (CTCL) subtype, commonly presents with limited-stage disease and is managed with skin-directed therapies. 1 Survival in these patients is measured in years to decades, with survival for those with <10% body surface area involvement comparable to that observed in age-matched subjects without CTCL. 1 In contrast, patients with advanced-stage disease, or limited-stage disease unresponsive to skin-directed therapies, require systemic therapy.…”

Hypopigmentation: mycosis fungoides in disguise

“…It has three clinical stages beginning with a patch phase, the stage at which this patient presented, progressing to a plaque phase and eventually to the tumour stage—which carries the worst prognosis 2. Early stage disease can easily be mistaken for multiple benign dermatoses 3…”