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目的:探讨脾高雪氏病的病理学特点、诊断、鉴别诊断及治疗。方法:收集2例脾脏高雪氏病,进行常规病理检查,伊红染色(HE)、免疫组化(Vim、CD68、CD163、LCA)染色及特殊染色(PAS),光镜观察,并结合国内外相关文献资料,分析其临床病理特点。结果:光镜下见脾脏内有高雪氏细胞。免疫组化染色Vim、CD68、CD163、LCA均弥漫性(+),S100(-),PAS染色胞质(+)。结论:脾脏高雪氏病是一种少见的常染色体隐性遗传性脑苷脂代谢障碍性疾病,有一定的病理学特征。应与Nieman-pick病、脾白血病、脾原发性淋巴瘤等鉴别。
Objective: To investigate the pathological features, diagnosis, differential diagnosis and treatment of splenic Gaucher disease. Methods: Two cases of Gaucher’s disease of spleen were collected and examined by routine pathological examination, HE stain, Vim, CD68, CD163, LCA and PAS, External related literature, analysis of its clinical and pathological features. Results: The light microscope, see the spleen have Gaucher cells. Immunohistochemical staining Vim, CD68, CD163, LCA were diffuse (+), S100 (-), PAS staining cytoplasm (+). Conclusions: Spleen Gaucher’s disease is a rare autosomal recessive cerebroside metabolic disorder with certain pathological features. Should be Nieman-pick disease, spleen leukemia, primary spleen lymphoma identification.