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Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers.
Australas J Dermatol 2016; 57(3):182-91AJ

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+Show Affiliations

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.Department of Dermatology, St Vincent's Hospital Department of Medicine, Melbourne, Victoria, Australia.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.Division of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.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.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

25988337

Citation

Hughes, Charlotte Fm, et al. "Mycosis Fungoides and Sézary Syndrome: Current Challenges in Assessment, Management and Prognostic Markers." The Australasian Journal of Dermatology, vol. 57, no. 3, 2016, pp. 182-91.
Hughes CF, Newland K, McCormack C, et al. Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers. Australas J Dermatol. 2016;57(3):182-91.
Hughes, C. F., Newland, K., McCormack, C., Lade, S., & Prince, H. M. (2016). Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers. The Australasian Journal of Dermatology, 57(3), pp. 182-91. doi:10.1111/ajd.12349.
Hughes CF, et al. Mycosis Fungoides and Sézary Syndrome: Current Challenges in Assessment, Management and Prognostic Markers. Australas J Dermatol. 2016;57(3):182-91. PubMed PMID: 25988337.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers. AU - Hughes,Charlotte Fm, AU - Newland,Kate, AU - McCormack,Christopher, AU - Lade,Stephen, AU - Prince,H Miles, Y1 - 2015/05/18/ PY - 2014/11/20/received PY - 2015/04/05/accepted PY - 2015/5/20/entrez PY - 2015/5/20/pubmed PY - 2017/4/25/medline KW - cutaneous lymphoma KW - mycosis fungoides KW - sezary syndrome SP - 182 EP - 91 JF - The Australasian journal of dermatology JO - Australas. J. Dermatol. VL - 57 IS - 3 N2 - 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. SN - 1440-0960 UR - https://www.unboundmedicine.com/medline/citation/25988337/Mycosis_fungoides_and_Sézary_syndrome:_Current_challenges_in_assessment_management_and_prognostic_markers_ L2 - https://doi.org/10.1111/ajd.12349 DB - PRIME DP - Unbound Medicine ER -