摘要
对1例结外鼻型NK/T细胞淋巴瘤临床表现、组织病理、免疫组化、EB病毒(Epstein Barr virus,EBV)原位杂交及基因重排进行分析。面部、四肢多发性红斑、结节及溃疡,组织病理呈血管炎改变,而免疫组化表达CD2(+)、CD3(+)、CD5(部分+)、CD7(部分+)、CD43(+)、CD4(+)、CD8(-)、CD56(+)、颗粒酶B(GrB)(+)、T细胞胞内抗原(TIA)-1(+)、间变性淋巴瘤激酶(ALK)(-)、CD20(-)、细胞特异性活化蛋白(PAX)-5(-)、Ki-67(LI约60%),EBV原位杂交阳性,骨髓穿刺未见异常,未检测到T细胞抗原受体(TCR)克隆性基因重排,鼻咽部未受累。诊断:结外鼻型NK/T细胞淋巴瘤。结外鼻型NK/T细胞淋巴瘤恶性程度高,病情发展迅速,对化疗不敏感,预后差。诊断有赖于临床表现结合组织病理、免疫表型检测、EBV原位杂交技术以及基因重排等。
To analyze the clinical manifestations, histopathology, immunohistochemical staining, in situ hybridization of Ep- stein-Barr virus and gene rearrangement in a case of extranodal NK/T-cell lymphoma, nasal type. The patient presented with multiple erythema, nodules and ulcers on the face and limbs. Histopathology displayed vaseulitic changes. Immunohistochemical staining showed positive for CD2, CD3, CD4, CD43, CD56, GrB, TIA-1 and partial positive for CD5 and CDT, 60% positive for Ki-67; CD8, ALK, CD20, and PAX-5 staining were negative. In situ hybridization of Epstein-Barr virus was positive. Bone marrow biopsy showed no abnormalities. TCR cloned gene rearrangement was undetected. Naso- pharynx was not affect- ed. The patient was diagnosed as extranodal NK/T-cell lymphoma, nasal type. This disorder is highly malignant and progresses rapidly with poor response to chemotherapy and poor prognosis. The diagnosis relies on clinical manifestations, histopathology, immunostaining, in situ hybridization of Epstein-Barr virus and gene rearrangement.
出处
《临床皮肤科杂志》
CAS
CSCD
北大核心
2013年第10期598-601,共4页
Journal of Clinical Dermatology
关键词
淋巴瘤
结外NK
T细胞
鼻型
lymphoma, extranodal NK/T-cell, nasal type
