Synonyms
Sezary sydrome, cutaneous T-cell lymphoma.
Definition
Mycosis fungoides (MF) and Sezary syndrome comprise a group of extranodal non-Hodgkin's lymphomas of T-cell origin.
Full Text
MF is a rare form of lymphoma, accounting for fewer than 1000 cases annually in the United States, or approximately 0.5% of all cases of lymphoma. Peak age at presentation is between 55 and 60 with 2:1 male:female ratio. The role of viruses has been proposed in the etiology of MF, and some studies have found HTLV I or HTLV II in tissue or blood from MF patients, however the significance of these findings remain uncertain.
MF presents as an indolent scaly erythematous eruption with patches or plaques similar to eczema or psoriasis. In some cases generalized erythroderma develops. Rarely patients have only pruritus without cutaneous lesions, but skin biopsies show infiltrates with clonal T-cells.
Sezary syndrome is considered to be the leukemic variant of MF which is characterized by generalized...
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References
Bunn PJ, Hoffman S, Norris D, et al (1994) Systemic therapy of cutaneous T-cell lymphoma. Annals of Internal Medicine 121:592
Olavarria E, Child F, Woolford A, et al (2001) T-cell depletion and autologous stem cell transplantation in the management of tumor stage mycosis fungoides with peripheral blood involvement. British Journal of Haematology 114:624
Siegel RS, Pandolfino T, Guitart J, et al (2000) Primary cutaneous T-cell lymphoma: review and current concepts. Journal of Clinical Oncology 18:2908
Zackheim HS, McCalmont TH (2002) Mycosis fungoides: The great imitator. Journal of the American Academy of Dermatology 47(6):914–8
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© 2004 Springer-Verlag
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(2004). Mycosis fungoides (MF). In: Moreland, L.W. (eds) Rheumatology and Immunology Therapy. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-29662-X_1930
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DOI: https://doi.org/10.1007/3-540-29662-X_1930
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-20625-5
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