Cutaneous extranodal NK/T-cell lymphoma: a clinicopathologic study of 5 patients with array-based comparative genomic hybridization

被引:42
作者
Berti, Emilio [1 ]
Recalcati, Sebastiano [2 ]
Girgenti, Valentina [2 ]
Fanoni, Daniele [2 ]
Venegoni, Luigia [2 ]
Vezzoli, Pamela [2 ]
机构
[1] Univ Milano Bicocca, Milan, Italy
[2] Univ Milan, Fdn IRCCS Ca Granda, Osped Maggiore Policlin,UO Dermatol, Dipartimento Anestesiol Terapia Intens & Sci Derm, Milan, Italy
关键词
NASAL-TYPE; T-CELL; PROGNOSTIC-FACTORS; TRANSPLANTATION; CLASSIFICATION; ORGANIZATION; NEOPLASMS; LEUKEMIA; SKIN;
D O I
10.1182/blood-2009-11-252957
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Extranodal natural killer/T-cell (ENK/T) lymphoma is a rare neoplasm, subcategorized into ENK/T-nasal (ENK/T-N) and ENK/T-nasal type (ENK/T-NT) lymphomas. ENK/T-NT lymphoma with initial presentation in the skin is known as primary cutaneous ENK/T-NT (PC-ENK/T-NT) lymphoma. The aim of this study was to investigate pathogenesis, genomic alterations, and prognosis of cutaneous ENK/T lymphomas to provide further insights into clinicopathologic features and genetic mechanism of lymphomagenesis. A retrospective case study of 5 white patients affected by ENK/T lymphoma (4 PC-ENK/T-NT and 1 ENK/T-N with cutaneous involvement) was performed. Most of the cases presented with multiple nodules and ulcerations localized on the extremities. A considerable percentage had disease in advanced stage with a 12-month survival rate of 40%. Genomic alterations were detected by array-based comparative genomic hybridization that showed gains of 1q, 7q and loss of 17p in the cases of PC-ENK/T-NT lymphomas and gain of 7q and loss of 9p, 12p, 12q in the case of ENK/T-N lymphoma. In conclusion, ENK/T lymphoma is a very aggressive entity, and, in our cases, the exclusively cutaneous presentation was not associated with a better prognosis. The results of our array comparative genomic hybridization analysis could be useful to better define the different ENK/T lymphoma subgroups with cutaneous involvement. (Blood. 2010; 116(2): 165-170)
引用
收藏
页码:165 / 170
页数:6
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