原发性免疫缺陷病尸检1例形态学观察及文献复习

Morphological changes of primary immunodeficiency with review of literature

目的 探讨原发性免疫缺陷病各脏器的病理组织特征及免疫器官在不同免疫状态下的形态、功能改变。方法 总结 1例原发性免疫缺陷病患儿的临床资料和尸检病理学特点 ,用抗酸、PAS、六胺银等特殊染色及AE1/AE3、CD1a、CD3、CD2 0、CD10、CD6 8、CD2 1、S 10 0、plasmacell、CD34等免疫组化染色协助诊断。结果 患儿胸腺、淋巴结、脾淋巴组织发育不良 ,T、B淋巴细胞数目都明显减少。特异性联合免疫缺陷导致肺脓肿 ,肝、脾、淋巴结结核 ,全身多脏器巨细胞病毒感染 ,肺卡氏肺囊虫等广泛、难以纠正的感染。结论 免疫系统成分多样 ,功能复杂 ,在机体不同免疫状态及外界不断刺激下 ,其形态学改变复杂。对于免疫系统疾病 ,需结合临床资料、形态学观察及必需的分子生物学技术进行诊断和鉴别诊断。

Objective To study the pathological features of p rimary immunodeficiency and the morphological and functional changes of the lymp h oid organs under different immune status. Methods Histopatholo gy and clinical course of one autopsy case of primary immunodeficiency was studi ed. Histochemistry for acid-fast bacilli stains, PAS, silver stain and immunohi st ochemistry for AE1/AE3, CD1a, CD3, CD20, CD10, CD68, CD21, S-100, plasma cell, and CD34 (EnVision method) were performed. Results Both T and B cell popu lations decreased. The thymic morphology showed dysplasia without the differenti a tion of Hassall's corpuscles. Combined deficiency of cellular and humoral immuni t y caused severe wide-spread infection, including lung abscess, tuberculosis in li ver, spleen and lymph nodes, CMV infection in most organs and Pnemocystis Cari ni i infection in lung. Conclusion The immune system has many c omponents and complex functions. Under different immune status and antigenic sti m ulation, the immune organs have complex morphological changes. Diagnosing the di se ases of immunity needs to combine the study of clinical information with the eva luati on of pathological features,and sometimes using the molecular biological techniq ues.