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Clinical immunology : the official journal of the Clinical Immunology Society v.183, 2017년, pp.112 - 120   SCI SCIE SCOPUS
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Maternal T and B cell engraftment in two cases of X-linked severe combined immunodeficiency with IgG1 gammopathy

Okano, Tsubasa (Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan ) ; Nishikawa, Takuro (Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan ) ; Watanabe, Eri (Laboratory of Diagnostic Medicine, Institute of Medical Science, The University of Tokyo, Tokyo, Japan ) ; Watanabe, Takashi (Laboratory for Integrative Genomics, RIKEN Center for Integrative Medical Sciences, Yokohama, Japan ) ; Takashima, Takehiro (Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan ) ; Yeh, Tzu-Wen (Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan ) ; Yamashita, Motoi (Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan ) ; Tanaka-Kubota, Mari (Department of Pediatrics and Developmental ) ; Miyamoto, Satoshi ; Mitsuiki, Noriko ; Takagi, Masatoshi ; Kawano, Yoshifumi ; Mochizuki, Yoshiki ; Imai, Kohsuke ; Kanegane, Hirokazu ; Morio, Tomohiro ;
  • 초록  

    Abstract X-linked severe combined immunodeficiency (X-SCID), caused by defects in the common gamma chain, is typically characterized by T and NK cell defects with the presence of B cells. T cell dysfunction and impaired class-switch recombination of B cells mean that patients typically have defects in class-switched immunoglobulins (IgG, IgA, and IgE) with detectable IgM. Here, we describe two patients with X-SCID with IgG1 gammopathy, in whom we identified maternal T and B cell engraftment. Exclusively, maternal B cells were found among the IgD − CD27 + class-switched memory B cells, whereas the patients' B cells remained naIve. In vitro stimulation with CD40L+IL-21 revealed that peripheral blood cells from both patients produced only IgG1. Class-switched maternal B cells had restricted receptor repertoires with various constant regions and few somatic hypermutations. In conclusion, engrafted maternal B cells underwent class-switch recombination and produced immunoglobulin, causing hypergammaglobulinemia in patients with X-SCID. Highlights Two cases of atypical X-SCID with IgG1 gammopathy had maternal T and B cell engraftment. Only maternal B cells differentiated to class-switched memory B cells and produced IgG1. Pre-class switched B cells engrafted then underwent class switch recombination in SCID patients. This is the first report to identify maternal B cell engraftment in B + SCID.


  • 주제어

    X-linked severe combined immunodeficiency .   IL2RG .   Maternal engraftment .   Hypergammaglobulinemia .   Gammopathy .   Multiple myeloma .   Allotype .   Memory B cell .   Somatic hypermutation .   Germinal center.  

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