国际儿科学杂志
國際兒科學雜誌
국제인과학잡지
INTERNATIONAL JOURNAL OF PEDIATRICS
2010年
4期
365-367,371
,共4页
自身免疫性%调亡%FAS基因%双阴性T细胞
自身免疫性%調亡%FAS基因%雙陰性T細胞
자신면역성%조망%FAS기인%쌍음성T세포
Autoimmunity%Apoptosis%FAS gene%Double negative T cell
自身免疫性淋巴细胞增生综合征是一种由于基因突变影响淋巴细胞程序性死亡或凋亡导致淋巴细胞自身稳定和正常免疫耐受障碍的罕见疾病.临床以淋巴细胞增生、自身免疫性疾病、恶性肿瘤为主要表现.此病发病机制和临床特点复杂,国内目前对此病了解甚少,易造成漏诊误诊,国内尚无此病个案报道.
自身免疫性淋巴細胞增生綜閤徵是一種由于基因突變影響淋巴細胞程序性死亡或凋亡導緻淋巴細胞自身穩定和正常免疫耐受障礙的罕見疾病.臨床以淋巴細胞增生、自身免疫性疾病、噁性腫瘤為主要錶現.此病髮病機製和臨床特點複雜,國內目前對此病瞭解甚少,易造成漏診誤診,國內尚無此病箇案報道.
자신면역성림파세포증생종합정시일충유우기인돌변영향림파세포정서성사망혹조망도치림파세포자신은정화정상면역내수장애적한견질병.림상이림파세포증생、자신면역성질병、악성종류위주요표현.차병발병궤제화림상특점복잡,국내목전대차병료해심소,역조성루진오진,국내상무차병개안보도.
Autoimmune lymphoproliferactive syndrome is a rare group disorders associated with self stability and immune tolerance of lymphocyte due to genetic mutation which affects the lymphocyte apoptosis .The clinical manifestations comprise lymphoproliferation, development of autoimmunity and malignancies.The pathogenesis and clinical features are complex ALPS were easily missed and no case had been reported until now in China.
