title: Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers.
abstract: Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires thorough history taking and physical examination, in combination with skin biopsy. In some cases flow cytometry, molecular studies and imaging are also required in order to diagnose and stage the disease. Staging is derived from the tumour-node-metastasis-blood classification and is currently our best attempt to stratify prognosis and hence guide management in this complex disease. Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. Management remains heavily guided by staging, such that patients with early-stage disease generally begin treatment with skin-directed or local therapies and those with advanced-stage disease have many treatment options, including chemotherapy, the use of biological agents, local and total body radiotherapy, as well as haematopoietic stem cell transplantation. Besides staging, many other patient-related factors influence the treatment strategy, particularly where symptom relief is paramount. There are many challenges remaining in the study of Mycosis fungoides and Sézary syndrome and, given the rarity of the disease, concerted worldwide efforts are required to conduct efficient and effective research.
authors.affiliation: Division of Haematology and Cancer Medicine, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Charlotte Fm
authors.initials: CF
authors.lastname: Hughes
authors.affiliation: Department of Dermatology, St Vincent's Hospital Department of Medicine, Melbourne, Victoria, Australia.
authors.forename: Kate
authors.initials: K
authors.lastname: Newland
authors.affiliation: Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Victoria, Australia.|Department of Dermatology, St Vincent's Hospital Department of Medicine, Melbourne, Victoria, Australia.
authors.forename: Christopher
authors.initials: C
authors.lastname: McCormack
authors.affiliation: Division of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.
authors.forename: Stephen
authors.initials: S
authors.lastname: Lade
authors.affiliation: Division of Haematology and Cancer Medicine, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: H Miles
authors.initials: HM
authors.lastname: Prince
mesh_terms: D000328:Adult; D001688:Biological Products / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000097:blood; D059248:Chemoradiotherapy / Q000379:methods; D003131:Combined Modality Therapy; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D011379:Prognosis; D018570:Risk Assessment; D012751:Sezary Syndrome / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D016019:Survival Analysis
chemical_list: D001688:Biological Products; D014408:Biomarkers, Tumor
keywords: cutaneous lymphoma; mycosis fungoides; sezary syndrome