Primary cutaneous acral CD8 positive T-cell lymphoma with extra-cutaneous involvement: A long-standing case with an unexpected progression

被引:25
作者
Alberti-Violetti, Silvia [1 ]
Fanoni, Daniele [1 ]
Provasi, Matteo [1 ,2 ]
Corti, Laura [1 ]
Venegoni, Luigia [2 ]
Berti, Emilio [1 ,2 ]
机构
[1] Fdn IRCCS Ca Granda Osped Maggiore Policlin, UOC Dermatol, Via Pace 9, I-20122 Milan, Italy
[2] Univ Milan, Dipartimento Fisiopatol Med Chirurg & Trapianti, Milan, Italy
关键词
acral CD8+lymphoma; bone involvement; cutaneous lymphoma; MYCOSIS-FUNGOIDES; PROLIFERATION; SITES; CLASSIFICATION; EXPRESSION; FEATURES; ENTITY; FACE; EAR;
D O I
10.1111/cup.13020
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
Primary cutaneous acral CD8+ T-cell lymphoma (acral CD8+ TCL) is a new provisional entity characterized by acral skin lesions and an indolent course. We describe an extraordinary case characterized by relapsed nodules with CD8+ cytotoxic infiltrates on the left ear. After 35 years, the skin lesions spread to other acral sites, and a mass with the same histological features as the other skin lesions appeared on the nose. Multiple courses of chemotherapy led to stable disease. Histological examinations carried out at different times showed the gradual transformation of the neoplastic cells, with an increased proliferation index. Genomic analysis revealed losses in the regions harboring the genes involved in cell cycle control. This is the first case of an acral CD8+ TCL with a very long history of indolent nodular lesions progressing to extra-cutaneous sites.
引用
收藏
页码:964 / 968
页数:5
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