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Titolo:
Immunologic reconstitution following bone marrow transplantation for X-linked hyper IgM syndrome
Autore:
Duplantier, JE; Seyama, K; Day, NK; Hitchcock, R; Nelson, RP; Ochs, HD; Haraguchi, S; Klemperer, MR; Good, RA;
Indirizzi:
Univ S Florida, All Childrens Hosp, Dept Pediat, Div Allergy & Immunol, StPetersburg, FL 33701 USA Univ S Florida St Petersburg FL USA 33701 nol, StPetersburg, FL 33701 USA Univ Washington, Sch Med, Dept Pediat, Seattle, WA 98195 USA Univ Washington Seattle WA USA 98195 , Dept Pediat, Seattle, WA 98195 USA Indiana Univ, Dept Hematol Oncol, Indianapolis, IN 46202 USA Indiana UnivIndianapolis IN USA 46202 Oncol, Indianapolis, IN 46202 USA
Titolo Testata:
CLINICAL IMMUNOLOGY
fascicolo: 3, volume: 98, anno: 2001,
pagine: 313 - 318
SICI:
1521-6616(200103)98:3<313:IRFBMT>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
CHILD;
Keywords:
bone marrow transplantation; immunodeficiency disease; high IgM, low IgG, and IgA; X-linked hyper IgM syndrome; immunization; T and B cells; CD40 ligand mutation(s); engraftment;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
10
Recensione:
Indirizzi per estratti:
Indirizzo: Duplantier, JE Univ S Florida, All Childrens Hosp, Dept Pediat, Div Allergy & Immunol, StPetersburg, FL 33701 USA Univ S Florida St Petersburg FL USA 33701 rg, FL 33701 USA
Citazione:
J.E. Duplantier et al., "Immunologic reconstitution following bone marrow transplantation for X-linked hyper IgM syndrome", CLIN IMMUNO, 98(3), 2001, pp. 313-318

Abstract

X-linked hyper IgM syndrome (XHIM), caused by mutations of the CD40 ligand(CD40L) gene, is characterized by recurrent bacterial and opportunistic infections, an increased incidence of autoimmunity and malignancies, and immunodeficiency due to abnormal T/B cell interaction. Because of poor long-term prognosis, bone marrow transplantation (BMT) has been proposed as an alternative treatment. An 8-month-old boy with XHIM and a splice site mutation of CD40L underwent BMT using a fully matched sibling donor. Markers of engraftment and immunologic reconstitution were measured serially. After BMT, activated T cells expressed functional CD40L, and genomic DNA obtained from circulating white cells contained predominantly wild-type CD40L sequences. Serum immunoglobulin levels including IgE and antibody responses to recall antigens normalized, and immunization with the T-cell-dependent neoantigen,bacteriophage phi X174, demonstrated amplification of the response and isotope switching. BMT provides a permanent cure for XHIM if a fully matched sibling donor is available and the procedure is performed before complications have occurred. (C) 2001 Academic Press.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/05/20 alle ore 13:11:20