原发皮肤血管内NK细胞淋巴瘤1例并文献复习

发布时间：2018-07-22 14:01

【摘要】：目的探讨皮肤血管内NK细胞淋巴瘤(intravascular NK cell lymphoma,IVNKL)的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析1例皮肤IVNKL患者的临床资料、病理学形态、免疫组化及EBER原位杂交染色结果,并复习相关文献。结果患者女性,47岁,临床表现为肩背部及双下肢皮肤多发暗红色质硬斑片及斑块。镜检:真皮及皮下组织内见较多扩张的小血管,血管腔内见中等到大的异型淋巴样细胞,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。免疫表型:肿瘤细胞CD3、CD56和TIA-1均阳性,Ki-67增殖指数70%。EBER分子原位杂交阳性。确诊后采用CHOP方案联合化疗2个疗程,目前仍在观察中。结论皮肤IVNKL是一种罕见的血管内淋巴瘤,预后较差且容易误诊,皮肤活检行病理检查是确诊的重要手段。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis, differential diagnosis, treatment and prognosis of intravascular NK cell lymphoma. Methods the clinical data, pathological morphology, immunohistochemistry and EBER in situ hybridization of a case of skin IVNKL were retrospectively analyzed. Results the patient was 47 years old, with multiple dark red hard spots and plaques on the shoulder, back and lower extremities. Microscopic examination: there were more dilated small blood vessels in dermis and subcutaneous tissue, medium to large dyslymphoid cells in vascular lumen, round nucleus, oval or irregular nucleus, deep staining of chromatin, cellulose like exudate in some vessels. Immunophenotype: CD3 + CD56 and TIA-1 were positive and Ki-67 proliferative index was 70%. EBER molecule was positive in situ hybridization. After diagnosis, chop regimen combined with chemotherapy was used for 2 courses, which is still under observation. Conclusion skin IVNKL is a rare intravascular lymphoma with poor prognosis and easy misdiagnosis.
【作者单位】：蚌埠医学院第一附属医院病理科;蚌埠医学院病理学教研室;上海交通大学附属第一人民医院内分泌科;皖南医学院附属弋矶山医院病理科;
【基金】：安徽省自然科学青年基金(1208085QH157)
【分类号】：R739.5

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