本帖最后由 pathology 于 2017-3-16 12:44 编辑
本周病例(2017.1.9—2017.1.15)
1、你考虑什么诊断?
结外NK/T细胞淋巴瘤,鼻型(Extranodal NK/T cell lymphoma, nasal type)
Histologically, they are angiocentric and angioinvasive with extensive coagulative necrosis and apoptosis. There also have perivascular and intravascular destructive infiltrates with fibrinoid changes of blood vessels even without invasion. The tumor cells are atypical small and large lymphoid cells with abundant pale or clear cytoplasm, irregular nuclear borders and immunoblasts. IHC shows positivity for CD2, CD3 (cytoplasmic CD3 epsilon, not membranous, in 56%), CD45+, CD56 (67-100%), TIA1, granzyme, EBER (96%). NK/T cell lymphomas are strongly associated with EBV, and EBV DNA titer is useful to monitor disease activity. The diagnosis should be questioned if negative for EBV by IHC and ISH.
CD3ε
CD20
CD56
EBER1/2-ISH
2、需要做什么鉴别诊断?
其他非霍奇金淋巴瘤、Wegener肉芽肿、淋巴瘤样肉芽肿、低分化癌、恶黑等
3、本病预后如何?
原发于鼻腔的结外NK/T细胞淋巴瘤总体生存率仅30-40%,早期病变对放疗敏感,同步放化疗的综合治疗模式明显改善其预后,5年生存率可达70%以上,而原发于鼻外的结外NK/T细胞淋巴瘤预后差。结外NK/T细胞淋巴瘤的预后因素受各方面影响:患者年龄、临床分期和治疗措施等均为结外NK/T细胞淋巴瘤患者的预后影响因素。EBV感染及Ki-67的表达同样可提示预后。研究发现EBV阴性患者的生存期明显高于EBV阳性患者,而Ki-67的高表达(≥65%)者临床生存期也明显缩短。不良预后因素包括:年龄>60岁,3-4期,p53错义突变,组织学呈大细胞免疫母细胞样多形性,LDH升高,皮肤/骨侵犯,区域淋巴结侵犯,Ki-67表达高,EBV-DNA>6.1×10^7/ml。
-----------------------------------------
本期病例比较典型,一旦考虑到此病,结合临床病史、组织学形态,辅以免疫组化和基因重排等则不难诊断。综合回答思路,月儿yuer、xhyong、fusuyong加一分,ARsenal.***、rewe鲜花鼓励!
希望大家一如既往的支持病理每周一题, |