日本顺天堂大学医学部免疫学教授奥村康和真贝洋一、理化学研究所国际研究新领域系统研究员泷尾扩士等合作,首次在世界上精制了 T 杀伤细胞和天然杀伤细胞担负杀伤靶细胞任务的穿孔蛋白(或穿孔素,Perforin),并以此成果为基础,克隆了 cDN...日本顺天堂大学医学部免疫学教授奥村康和真贝洋一、理化学研究所国际研究新领域系统研究员泷尾扩士等合作,首次在世界上精制了 T 杀伤细胞和天然杀伤细胞担负杀伤靶细胞任务的穿孔蛋白(或穿孔素,Perforin),并以此成果为基础,克隆了 cDNA。这项研究是与住友电工公司共同进行的。已由公司申请了专利。这项成果除已在《Na-展开更多
We analysed data of 96 infants (under 1 year of age) with haemophagocytic lymp hohistiocytosis (HLH) from the registry of an HLH study conducted during 1986-2 002 in Japan. The cases were classified into five groups. ...We analysed data of 96 infants (under 1 year of age) with haemophagocytic lymp hohistiocytosis (HLH) from the registry of an HLH study conducted during 1986-2 002 in Japan. The cases were classified into five groups. The diagnosis of famil ial HLH (FHL) as group 1 (n =27) wasmade with positive family history and/or rec ent molecular test for perforin and Munc13-4 mutations. Neonatal enterovirus-o r herpes simplex virus-associated HLH as group 2a (n =7), Epstein-Barr virus- associated HLH (n =12) as group 2b, adenovirus-or cytomegalovirus-associated H LH as group 3 (n =9)were mostly diagnosed by viral isolation or by the detection of viral genome. Juvenile rheumatoid arthritis-associated macrophage activatio n syndrome was classified as group 4 (n =4) and the remaining without known trig gers as group 5 (n =37). The peak onset age was 1-2 months for group 1, 1-2 we eks for group 2a, 12 months for group 2b, none for group 3, 9 months for group 4 and 2 months for group 5. Future novel diagnostic measures are required to defi ne the precise nature of HLH in group 5. Conclusion:These data may provide usefu l information for neonatologists/ paediatricians in the differential diagnosis o f haemophagocytic lymphohistiocytosis in early infancy.展开更多
文摘日本顺天堂大学医学部免疫学教授奥村康和真贝洋一、理化学研究所国际研究新领域系统研究员泷尾扩士等合作,首次在世界上精制了 T 杀伤细胞和天然杀伤细胞担负杀伤靶细胞任务的穿孔蛋白(或穿孔素,Perforin),并以此成果为基础,克隆了 cDNA。这项研究是与住友电工公司共同进行的。已由公司申请了专利。这项成果除已在《Na-
文摘We analysed data of 96 infants (under 1 year of age) with haemophagocytic lymp hohistiocytosis (HLH) from the registry of an HLH study conducted during 1986-2 002 in Japan. The cases were classified into five groups. The diagnosis of famil ial HLH (FHL) as group 1 (n =27) wasmade with positive family history and/or rec ent molecular test for perforin and Munc13-4 mutations. Neonatal enterovirus-o r herpes simplex virus-associated HLH as group 2a (n =7), Epstein-Barr virus- associated HLH (n =12) as group 2b, adenovirus-or cytomegalovirus-associated H LH as group 3 (n =9)were mostly diagnosed by viral isolation or by the detection of viral genome. Juvenile rheumatoid arthritis-associated macrophage activatio n syndrome was classified as group 4 (n =4) and the remaining without known trig gers as group 5 (n =37). The peak onset age was 1-2 months for group 1, 1-2 we eks for group 2a, 12 months for group 2b, none for group 3, 9 months for group 4 and 2 months for group 5. Future novel diagnostic measures are required to defi ne the precise nature of HLH in group 5. Conclusion:These data may provide usefu l information for neonatologists/ paediatricians in the differential diagnosis o f haemophagocytic lymphohistiocytosis in early infancy.
