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CD25 + folliculotropic S ézary syndrome with CD30 + large cell transformation
Author(s) -
Jang Min Soo,
Kang Dong Young,
Han Sang Hwa,
Park Jong Bin,
Kim Sang Tae,
Suh Kee Suck
Publication year - 2014
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/ajd.12000
Subject(s) - mycosis fungoides , medicine , cd30 , lymphoma , cutaneous lymphoma , cutaneous t cell lymphoma , dermatology , aggressive lymphoma , pathology , rituximab
Folliculotropic S ézary syndrome is a rare and unique variant of cutaneous T ‐cell lymphoma ( CTCL ) characterised by both follicular and leukaemic involvement of mycosis fungoides ( MF ). It is associated with a more aggressive clinical course and fatal outcomes. Large cell transformation ( LCT ) of mycosis fungoides/ S ézary syndrome is also associated with an aggressive disease course and shortened survival, requiring an intensive therapeutic approach. This report describes a case of folliculotropic S ézary syndrome with CD30 + LCT . Most of the larger lymphocytes in the lesions were positive for CD25 , the expression of which is associated with advanced CTCL . In addition, we review the literature on this unusual CTCL and provide evidence that this entity represents a distinct clinicopathological entity occasionally associated with extracutaneous involvement and LCT .