脾高雪氏病误诊为血管肉瘤1例并文献复习

目的报告1例脾高雪氏病误诊病例。方法应用光镜对1例原诊断脾血管肉瘤病例，重新取材进行形态学观察，并进一步做PAS和免疫组化染色。结合文献对其临床病理特点，鉴别诊断和误诊原因进行探讨。结果脾高雪氏病临床无特殊症状。光镜具有特征性的细胞，PAS、CD68呈阳性，CD34呈阴性。结论脾高雪氏病是一种脂质代谢障碍引起的非肿瘤病变，与血管肉瘤有本质上的区别。PAS染色、免疫组化组织细胞标记及血管内皮标记具有辅助诊断意义。

Misdiagnosis Gaucher''''s disease of spleen as hemangiosarcoma case report and review of the literature

Objective To report a case with a misdiagnosed spl en ic disease. Methods Splenic specimens from a case with a previously diagnosed splenic.hemangiosarcoma was obtained again, and then observ ed under light microscope. In addition, PAS staining and immunohistochemical sta ining were also performed on these specimens. In combination with related litera tures, the pathological characteristic of specimens, the differential diagnosis of this disease as well as the misdiagnosed cause were finally discussed. Results Clinical manifestations of Gaucher's disease of spleen are nonspecific. However, specific cells in splenic specimens can be seen by us ing light microscope. Moreover, PAS and CD68 staining are positive but CD34 stai ning negative. Conclusion As a benign disease, Gaucher's disease of spleen is characterized by dyslipidosis and completely different to s plenic hemangiosarcoma. The PAS staining and the detection of two molecular make rs, CD68 and CD34 are helpful in identifying this disease.