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目的:报告2例先天性胸腺发育不良病例,以提高对该病的认识。方法:通过尸体解剖,应用组织病理学技术方法,观察胸腺、脾脏、淋巴结及骨髓等组织学改变,选择免疫组化(CD20、CD3、CD5、CD45RA、CD45RO、EBV)和特殊染色进行观察。结果:淋巴器官发育不良,胸腺缺乏皮髓分化;脾组织内淋巴细胞稀疏,脾小体鞘区淋巴细胞少;淋巴结淋巴滤泡缺乏;肠壁、阑尾及咽部粘膜等淋巴组织淋巴细胞稀疏;骨髓造血细胞无明显异常。结论:由于先天性胸腺发育不良,患儿存在原发性免疫缺陷,招致反复难治性感染。如能做出早期正确诊断,对症治疗的同时,可考虑胚胎胸腺移植及胸腺素治疗,以改善患者免疫缺陷状态。
Objective: To report 2 cases of congenital thymus dysplasia to improve the understanding of the disease. Methods: Histological changes of thymus, spleen, lymph nodes and bone marrow were observed by autopsy and histopathological techniques. Immunohistochemistry (CD20, CD3, CD5, CD45RA, CD45RO, EBV) and special staining were used to observe the histological changes. Results: Lymphatic organs were poorly developed, there was a lack of myeloid differentiation in the thymus, sparse lymphocytes in the spleen tissues, few lymphocytes in the splenic body, lymphocyte follicles in the lymph nodes, sparse lymphocytes in the intestinal wall, appendix and pharynx mucosa. Bone marrow hematopoietic cells without obvious abnormalities. Conclusion: Due to congenital thymus dysplasia, children with primary immune deficiency, leading to repeated refractory infections. If you can make the early correct diagnosis, symptomatic treatment, you can consider the embryo thymus transplantation and thymosin treatment to improve the patient’s immune deficiency status.