脾高雪病的临床病理及免疫组化分析
作者：赵强 周建华
单位：赵强(湖南衡阳医学院第一附属医院病理科 421001)；周建华(湖南医科大学病理教研室)
关键词：高雪病；脾脏；临床病理；免疫组化
诊断病理学杂志000205摘 要：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，CD68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-葡萄糖苷酶活性的测定对本病确诊具有十分重要的意义。
分类号：R589.2 文献标识码：A
文章编号：1007-8096(2000)02-0093-03
Clinicopathologic and Immunohiatochemical Studies of Gaucher’s Disease in the Spleen
Zhao Qiang ,Zhou Jianghua
(Department of Pathology，The First Affiliated Hospital，Heng Yang Medical College，Hengyang 421001)
Abstract：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.
Keywords：Gaucher’s disease Spleen Clinicopathology Immunohistochemistry
参考文献：
[1]陈文杰，陈辉树.造血系统疾病临床病理学.北京：北京医科大学联合出版社,1997:73
[2]Fleshner PR，Arthur H，Gregory A，et al.A 27-year Experience with splenectomy for Gaucher’s Disease.The Amerjcan.journal of surgery，1991:161:74-75
[3]刘彤华主编.诊断病理学.北京：人民卫生出版社.1995:677
[4]袁孝宾，孙 琰.高雪氏病研究进展，国外医学生理，病理科学与临床分册,1994;14:91-92
[5]张为民，施惠平，郭玉凤，等.高雪氏病的诊断及产前诊断.中华医学遗传学杂志,1992;9:2-3
收稿日期：1999-09-02
修稿日期：2000-03-02, 百拇医药
单位：赵强(湖南衡阳医学院第一附属医院病理科 421001)；周建华(湖南医科大学病理教研室)
关键词：高雪病；脾脏；临床病理；免疫组化
诊断病理学杂志000205摘 要：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，CD68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-葡萄糖苷酶活性的测定对本病确诊具有十分重要的意义。
分类号：R589.2 文献标识码：A
文章编号：1007-8096(2000)02-0093-03
Clinicopathologic and Immunohiatochemical Studies of Gaucher’s Disease in the Spleen
Zhao Qiang ,Zhou Jianghua
(Department of Pathology，The First Affiliated Hospital，Heng Yang Medical College，Hengyang 421001)
Abstract：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.
Keywords：Gaucher’s disease Spleen Clinicopathology Immunohistochemistry
参考文献：
[1]陈文杰，陈辉树.造血系统疾病临床病理学.北京：北京医科大学联合出版社,1997:73
[2]Fleshner PR，Arthur H，Gregory A，et al.A 27-year Experience with splenectomy for Gaucher’s Disease.The Amerjcan.journal of surgery，1991:161:74-75
[3]刘彤华主编.诊断病理学.北京：人民卫生出版社.1995:677
[4]袁孝宾，孙 琰.高雪氏病研究进展，国外医学生理，病理科学与临床分册,1994;14:91-92
[5]张为民，施惠平，郭玉凤，等.高雪氏病的诊断及产前诊断.中华医学遗传学杂志,1992;9:2-3
收稿日期：1999-09-02
修稿日期：2000-03-02, 百拇医药