摘要
本文报告了3例临床较少见的原发性免疫缺陷病。例1为选择性 IgM 缺乏症;例2为先天性胸腺发育不全,例3为湿疹、血小板减少伴免疫缺陷综合征。按原发性免疫缺陷病的传统分类,例1属体液免疫缺陷病,例2属细胞免疫缺陷病,例3属联合免疫缺陷病。随着分子生物学的发展,T 细胞在免疫调节中的作用已为人们所公认,现认为上述3例均属原发性 T 细胞功能缺陷所致的 Ig 缺乏症。作者对该病的病因、遗传方式、临床表现及治疗进行了讨论。
Three clinically rare cases of primary immunodeficiency were reported.The firstcase was of selective IgM deficiency;the second is Di George Syndrome;and thethird is Wiskott-Aldrich Syndrome.In accordance with the classical classification of primary immunodeficiency,thefirst was humoral immunodefieiency;the second was cell-mediated immunodeficiencyand the third was combined immunodeficiency.With the development of the molecu-lar biology,observation on T cell function in immuno regulation was now possible.All three cases were considered originating from primary T-cell function deficiencycausing Ig deficiency.The causes,hereditary,clinical manifestations and treatmentof the disease were discussed.
出处
《天津医药》
CAS
1991年第8期472-474,共3页
Tianjin Medical Journal
