窦组织细胞增生伴巨大淋巴结病累及鼻腔临床病理观察

Sinus histiocytosis with massive lymphadenopathy:a clinicopathological study

目的探讨窦组织细胞增生伴巨大淋巴结病(SHML)的临床病理特征。方法对1例颈部、鼻腔同时发生SHML的病例进行临床病理分析及免疫组化研究,并进行文献复习。结果颈部淋巴结SHML临床表现为双侧颈部无痛性巨大型淋巴结肿大,镜下病变淋巴结淋巴窦扩张,淋巴窦内充盈淋巴细胞、浆细胞及大量组织细胞,并可见特征性吞噬淋巴细胞、浆细胞或红细胞的组织细胞。鼻腔SHML的临床表现为鼻塞、鼻出血等。镜下病变主要由梭形细胞组成,伴有大量炎细胞浸润,伸入运动不显著。免疫组化显示颈部淋巴结和鼻腔组织细胞S-100和CD68(+),CD1α(-)。结论 SHML可累及鼻腔,诊断主要依赖病理组织学和免疫组化。

Objective To evaluate the clinicopathologic features of sinus histiocytosis with massive lymphadenopathy.Methods A case of sinus histiocytosis with massive lymphadenopathy（SHML） in lymph nodes of neck as well as involving nasal cavity was examined with conventional histopathological and immunohistochemical stains.Results The clinical symptoms of the patient with SHML were painless enlargement of the lymph nodes of neck.When SHML involving nasal cavity,the symptoms were nasal obstruction,epistaxis and so on.Histologically,SHML＇s histopathological characteristic of lymph nodes was the dilatation of lymph sinus and filled with lymphocytes,plasma cells,neutrophils and eosinophils.The histiocytes with engulfment of lymphocytes and red cells were prominent.SHML in nasal cavity showed a spindle-cell population associated with a diffuse and florid mixed inflammatory cell infiltrate of neutrophils,plasma cells and lymphocytes,lymphophagocytosis（emperipolesis） was subtle.Immunohistochemically,the histiocytes were positive for S-100 and CD68.Conclusion Concurrence of SHML in nosal cavity and in lymph nodes of neck is rare.Its diagnosis depends on histopathological and immunohistochemical findings.