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Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014
Online ISSN 1827-1820
Scarisbrick J. J.
Skin Tumour Unit, St Thomas Hospital, London, UK
Little is known about the pathogenesis of mycosis fungoides and Sézary syndrome. Specific translocations involving up-regulation of oncogenes, which characterise nodal lymphomas and leukaemias, have not been identified although abnormalities of the P53 tumour suppressor gene have been detected in advanced disease. Loss of heterozygosity (LOH) is a mechanism of tumour suppressor gene inactivation and such studies may be used to screen for sites of candidate tumour suppressor genes. Allelic loss was present in 45% of patients with mycosis fungoides and 67% with Sézary syndrome. Rates or loss over 10% were detected in mycosis fungoides on 9p (16%), 10q (12%), 1p (10%) and 17p (10%). Allelic losses in 1p and 9p were found in all stages of mycosis fungoides and on 17p and 10q in advanced disease. In Sézary syndrome higher rates of LOH were detected; 9p (46%), 17p (42%), 2p (12%), 6q (7%) and 10q (12%). There was no loss detected on 1p. A further study by our group using comparative genomic hybridization analysis (CGH) showed chromosome imbalances in 19 of 34 cutaneous T-cell lymphoma (CTCL) cases (56%). The most frequent losses involved chromosomes were 1p (38%), 17p (21%), 10q (15%) and 19 (15%). These studies provide information on the likely sites of tumour suppressor genes involved in the pathogenesis of CTCL. Patterns of allelic loss suggest the involvement of genes on 1p and 9p in early disease whilst genes on 10q and 17p may be important in disease progression. Candidate tumour suppressor genes include the PTEN (10q23.3), Fas (10q24.1), P15 (9p21), P16 (9p21), P73 (1p36), P18 (1p32) and TAL-1 (1p32) genes.