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A case of hydroa vacciniforme-like primary cutaneous NK/T-cell lymphoma
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- Author:
- No author available
- Journal Title:
- CHINESE JOURNAL OF DERMATOLOGY
- Issue:
- 12
- DOI:
- 10.3760/j.issn:0412-4030.2007.12.004
- Key Word:
- 淋巴瘤;杀伤细胞,天然;疱疹病毒4型,人;爱泼斯坦巴尔病毒感染
Abstract： 患儿男,11岁,面部、手臂出现红斑、水疱4年,四肢肿块、溃疡4个月.逐渐感觉乏力,有不规则间歇发热.体检:体温37.7℃.全身淋巴结无明显肿大,口腔,颜面和鼻部皮肤片样轻度浸润的粉红色斑片,四肢见大小不等的暗红色浸润性结节和肿块,结节直径2.0～18 cm不等,部分表面有坏死和黑色结痂,较大的结节表面形成溃疡,坏死性溃疡深达肌肉并有白色脓性分泌物.皮损边界清楚,质地较硬,有压痛,部分结节周围见到卫星样小结节.取自大腿的皮损病理检查发现,表皮真皮全层至皮下可见大量形态不一、中等大小的肿瘤细胞浸润,肿瘤细胞胞质清晰,核大,扭曲状,染色质丰富,细胞呈巢或散在,可见围管样分布;真皮和皮下见碎核和反应性组织细胞浸润,肿瘤细胞浸入毛囊和外泌汗腺.免疫组化:胞质CD3在核周区可见阳性染色,CD56、CD45RO和TIA-1强阳性(+++),部分肿瘤细胞CD30、CD8和Ki 67阳性(++).EB病毒编码小核RNA(EBER)阳性,TCR-γ基因重排阳性.最后诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤,这是1例罕见的原发于皮肤长达51个月的进展缓慢的病例.
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