脾高雪病的临床病理及免疫组化分析

Clinicopathologic and Immunohiatochemical Studies of Gaucher's Disease in the Spleen

目的 阐明高雪病的临床病理形态特征 ,为临床诊断提供确切依据。方法 对 5例脾高雪病手术切除标本和会诊病例采用光镜组织形态学观察 ,辅以PAS、免疫组化染色 ,结合文献对本病临床病理特点进行探讨。结果 高雪病患者具有无痛性脾肿大 ,伴有轻～中度贫血 ,光镜下见高雪细胞胞浆呈淡红色皱纹样、PAS强阳性反应染色 ,CD6 8强阳性 ,Lysozyme阳性。结论 本病术前容易误诊 ,慢性巨脾症患者应考虑本病的可能 ;免疫组化标记显示属组织细胞源性疾病 ;PAS染色、酸性磷酸酶染色及血液 β

Objective To study pathomorphologic characteristics of spleen Gaucher's disease to provide more information for clinical diagnosis. Methods Immunohistochemistry, PAS and H E staining were used for studying tissue samples from 5 patients with spleen. Gaucher's disease. Results The patients presented painless splenomegalia and mild moderate anemia. Gaucher's cells had abundant cytoplasm containing distinct striations and were strong positive for PAS and CD68, and weak positive for lysozyme. Conclusion Misdiagnosis of Gaucher's disease usually happens before surgical move of the spleen. The diagnosis should be considered for the patient with chronic megalosplenia. Pathologic analyses, PAS and acid phosphatase stainings and detection of glucosaccharase activity in blood are very important for diagnosis of Gaucher's disease.