Premium
Cutaneous T ‐cell lymphoma: 2016 update on diagnosis, risk‐stratification, and management
Author(s) -
Wilcox Ryan A.
Publication year - 2016
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24233
Subject(s) - medicine , mycosis fungoides , lymphoma , oncology , cutaneous t cell lymphoma , t cell lymphoma , disease , stage (stratigraphy) , chemotherapy , transplantation , autologous stem cell transplantation , dermatology , paleontology , biology
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, 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 before escalating therapy to include systemic, single‐agent chemotherapy. In highly‐selected patients, allogeneic stem‐cell transplantation may be considered, as this may be curative in some patients. Am. J. Hematol. 91:151–165, 2016. © 2015 Wiley Periodicals, Inc.