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

Am J Hematol. 2011 Nov;86(11):928-48. doi: 10.1002/ajh.22139.

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.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use*
  • Combined Modality Therapy / methods*
  • Diphtheria Toxin
  • Disease Management
  • Histone Deacetylase Inhibitors
  • Humans
  • Immunohistochemistry
  • Interleukin-2
  • Lymphoma, T-Cell, Cutaneous* / diagnosis
  • Lymphoma, T-Cell, Cutaneous* / immunology
  • Lymphoma, T-Cell, Cutaneous* / pathology
  • Lymphoma, T-Cell, Cutaneous* / therapy
  • Mycosis Fungoides* / diagnosis
  • Mycosis Fungoides* / immunology
  • Mycosis Fungoides* / pathology
  • Mycosis Fungoides* / therapy
  • Neoplasm Staging
  • Prognosis
  • Recombinant Fusion Proteins
  • Risk Assessment
  • Sezary Syndrome* / diagnosis
  • Sezary Syndrome* / immunology
  • Sezary Syndrome* / pathology
  • Sezary Syndrome* / therapy
  • Skin / immunology
  • Skin / pathology*
  • Stem Cell Transplantation*
  • T-Lymphocytes / immunology
  • T-Lymphocytes / pathology*
  • Transplantation, Homologous
  • Treatment Outcome

Substances

  • Antineoplastic Agents
  • Diphtheria Toxin
  • Histone Deacetylase Inhibitors
  • Interleukin-2
  • Recombinant Fusion Proteins
  • denileukin diftitox