|Cancer : Mycosis fungoides/Sezary's syndrome|
|Cancer Name||Mycosis fungoides/Sezary's syndrome|
|Clinics and Pathology|
|Epidemiology||This is the most common form of cutaneous T-cell lymphoma. The annual incidence is around 0,3 cases per 100.000 in western countries. The median age at diagnosis is between 55 and 60 years, with a 2/1 male-to-female ratio.
|Pathology||The tumor cell is a small lymphocyte with cerebriform nucleus, clumped chromatin and inconspicuous nucleoli. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabsecsses is the hallmark of the disease.
|Treatment||Phototherapy, radiation therapy and alpha interferon are the mainstay of treatment of cutaneous disease. Chemotherapy using various regimens was employed in cases displaying disseminated disease and in Sezary's syndrome with limited success.
|Prognosis||The clinical stage is the most important indicator. Patient with limited cutaneous disease have an excellent prognosis. Patient with cutaneous tumours, generalized erythroderma, with Sezary's syndrome or extracutaneous disease usually have a short survival, ranging from 1 to 4 years.
|Title||Amplification and overexpression of JUNB is associated with primary cutaneous T-cell lymphomas.|
|Authors||Mao X, Orchard G, Lillington DM, Russell-Jones R, Young BD, Whittaker SJ.|
|Citations||Blood 2003; 101: 1513-1519.|
|Title||Recurring structural chromosome abnormalities in peripheral blood lymphocytes of patients with mycosis fungoides/Sezary syndrome.|
|Authors||Thangavelu M, Finn WG, Yelavarthi KK, Roenigk HH Jr, Samuelson E, Peterson L, Kuzel TM, Rosen ST.|
|Citations||Blood 1997; 89: 3371-3377.|
|Title||Molecular cytogenetic detection of chromosomal breakpoints in T-cell receptor gene loci.|
|Authors||Gesk S, Martin-Subero JI, Harder L, Luhmann B, Schlegelberger B, Calasanz MJ, Grote W, Siebert R.|
|Citations||Leukemia 2003; 17: 738-745.|
|Title||Molecular cytogenetic characterization of Sezary syndrome.|
|Authors||Mao X, Lillington DM, Czepulkowski B, Russell-Jones R, Young BD, Whittaker S.|
|Citations||Genes Chromosomes Cancer 2003; 36: 250-260.|
|Title||Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sezary syndrome and mycosis fungoides.|
|Authors||Mao X, Lillington D, Scarisbrick JJ, Mitchell T, Czepulkowski B, Russell-Jones R, Young B, Whittaker SJ.|
|Citations||Br J Dermatol 2002; 147: 464-475.|
|Title||Cutaneous T-cell lymphomas|
|Authors||Kim Y, Hoppe RT.|
|Citations||In: Magrath I (Ed) The non Hodgkin's lymphomas 2nd edition. Pp 907-926; Arnold, London 1997.|
|Source and Citation|
|Source||Atlas of Genetics and Cytogenetics in Oncology and Haematology|
|Citation|| Cuneo A, Castoldi GL . Mycosis fungoides/Sezary's syndrome. Atlas Genet Cytogenet Oncol Haematol. May 2005 .
URL : http://AtlasGeneticsOncology.org/Anomalies/MycosFungID2039.html