Altered germinal center reaction and abnormal B cell peripheral maturation in PI3KR1-mutated patients presenting with HIGM-like phenotype

被引:55
作者
Lougaris, Vassilios [1 ,2 ]
Faletra, Flavio [3 ]
Lanzi, Gaetana [4 ,5 ]
Vozzi, Diego [3 ]
Marcuzzi, Annalisa [3 ]
Valencic, Erica [3 ]
Piscianz, Elisa [3 ]
Bianco, Anna Monica [3 ]
Girardelli, Martina [3 ]
Baronio, Manuela [1 ,2 ]
Loganes, Claudia [3 ]
Fasth, Anders [6 ]
Salvini, Filippo [7 ]
Trizzino, Antonino [8 ]
Moratto, Daniele [4 ,5 ]
Facchetti, Fabio [9 ]
Giliani, Silvia [4 ,5 ]
Plebani, Alessandro [1 ,2 ]
Tommasini, Alberto [3 ]
机构
[1] Univ Brescia, Pediat Clin, Dept Clin & Expt Sci, Piazzale Spedali Civili 1, I-25123 Brescia, Italy
[2] Univ Brescia, Inst Mol Med A Nocivelli, Dept Clin & Expt Sci, I-25123 Brescia, Italy
[3] IRCCS Burlo Garofolo, Inst Maternal & Child Hlth, Trieste, Italy
[4] Univ Brescia, Inst Mol Med A Nocivelli, Dept Mol & Translat Med, Lab Genet Disorders Childhood, I-25123 Brescia, Italy
[5] Univ Brescia, Dept Pathol, Dept Mol & Translat Med, Lab Genet Disorders Childhood, I-25123 Brescia, Italy
[6] Univ Gothenburg, Sahlgrenska Acad, Inst Clin Sci, Dept Pediat, Gothenburg, Sweden
[7] Univ Milan, San Paolo Hosp, Dept Pediat, Milan, Italy
[8] ARNAS CIVICO, Dept Pediat Oncol & Hematol, Palermo, Italy
[9] Univ Brescia, Dept Mol & Translat Med, Pathol Unit, Brescia, Italy
关键词
PIK3R1; Hyper-IgM syndrome; B cells; Germinal Center (GC) reaction; HUMAN IMMUNODEFICIENCY; ACTIVATION; MUTATIONS;
D O I
10.1016/j.clim.2015.04.014
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
引用
收藏
页码:33 / 36
页数:4
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