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目的探讨皮肤结外鼻型血管内NK/T细胞淋巴瘤的临床病理特征、诊断和鉴别诊断、治疗及预后。方法回顾性分析1例皮肤结外鼻型血管内NK/T细胞淋巴瘤患者的临床资料、组织病理形态、免疫组化染色及原位杂交染色。结果光镜下皮肤附属器周围、血管周围和血管内可见中等大小的异型淋巴样细胞,肿瘤细胞胞质比较丰富、淡染,细胞核圆形、卵圆形或不规则,染色质深染,部分血管内可见纤维素样渗出物。免疫组化显示肿瘤细胞CD3和粒酶B(+),CD56部分(+),CD5和CD8散在(+),CD4、CD20和MPO(-);CD31显示大部分肿瘤细胞位于血管内;Ki-67阳性率>50%。EBER分子原位杂交(+)。结论皮肤结外鼻型血管内NK/T细胞淋巴瘤是一种罕见的血管内淋巴瘤,临床和病理均易与皮肤血管炎性病变混淆,因此掌握其临床病理特征对该病的诊断和鉴别诊断具有重要意义。
Objective To investigate the clinicopathological characteristics, diagnosis, differential diagnosis, treatment and prognosis of extranodal nasal type NK / T cell lymphoma. Methods The clinical data, histopathology, immunohistochemistry and in situ hybridization of one patient with extranodal nasal type NK / T cell lymphoma were retrospectively analyzed. Results Under the light microscope, allograft lymphoid cells of moderate size around the perivascular appendages, blood vessels and blood vessels were found. The tumor cells were rich in cytoplasm, lightly stained, round nucleus, ovoid or irregular, chromatin stained, part Vascular visible cellulose-like exudate. Immunohistochemistry showed that the tumor cells were scattered (+), CD4, CD20 and MPO (-) in the CD3 and granzyme B (+), CD56 (+), CD5 and CD8; CD31 showed that most of the tumor cells were located in the blood vessels; 67 positive rate> 50%. EBER molecular in situ hybridization (+). Conclusions The extranodal nasal type NK / T cell lymphoma is a rare intravascular lymphoma which is easily confused with cutaneous vasculitis in clinic and pathology. Therefore, to master the clinical and pathological features of the disease, Diagnosis is of great importance.