PMID: 8942778Nov 30, 1996Paper

In-utero transplantation of parental CD34 haematopoietic progenitor cells in a patient with X-linked severe combined immunodeficiency (SCIDXI)

Lancet
G S WenglerA G Ugazio

Abstract

X-linked severe combined immunodeficiency (SCIDXI) is an inherited immune defect which leads to death in infancy from severe infections. The defect is caused by mutations of the IL-2RG gene that encodes for the common gamma chain shared by several cytokine receptors. The disease is characterised by lack of T and NK cells with normal numbers of B cells. SCIDXI can be cured by bone marrow transplantation (BMT) or prevented by abortion after prenatal diagnosis. A male fetus was diagnosed as having SCIDXI by molecular, immunophenotypic, and functional analyses. The fetus was injected intraperitoneally under ultrasound guidance with CD34 haematopoietic progenitor cells purified from paternal bone marrow and T-cell depleted by E rosetting. Chimerism analysis was by HLA-DQ alpha typing and gamma-chain staining on cord blood. A healthy 3.6 kg boy was delivered by caesarean section at 38 weeks of gestation with no clinical or laboratory signs of graft-versus-host disease. Engraftment of donor-derived CD2 cells was found at birth. At 3.5 months of age the infant is well and his T-cell counts and function are normal. In-utero transplantation of haematopoietic progenitor cells allowed immune reconstitution of a fetus with SCIDXI and may be...Continue Reading

Citations

Nov 25, 2000·Clinical and Experimental Immunology·A Fischer
Oct 1, 2005·Proceedings of the National Academy of Sciences of the United States of America·Annalisa FrattiniAnna Villa
Aug 23, 2001·Archives of Disease in Childhood. Fetal and Neonatal Edition·L KaneA J Cant
Apr 18, 2000·Journal of Clinical Pathology·A M Jones, H B Gaspar
Jan 20, 2011·The Journal of Clinical Investigation·Amar NijagalTippi C MacKenzie
Sep 23, 2010·Laboratory Hematology : Official Publication of the International Society for Laboratory Hematology·Faris Q AlenziRichard K H Wyse
Feb 11, 2011·Journal of Visualized Experiments : JoVE·Amar NijagalTippi C Mackenzie
Jan 1, 2009·Mediterranean Journal of Hematology and Infectious Diseases·Maria Concetta Renda, Aurelio Maggio
Oct 20, 2009·Early Human Development·Arnalda LanfranchiGaetano Chirico
Nov 19, 2004·Transplantation·Mohamed E MoustafaEwa Carrier
Jan 18, 2007·Fetal Diagnosis and Therapy·Scott M PetersenKarin J Blakemore
Jan 27, 2019·Blood Advances·Alessandra MagnaniMarina Cavazzana
Mar 16, 2019·Current Stem Cell Reports·Amir Alhajjat, Aimen Shaaban
Apr 29, 1998·Current Opinion in Obstetrics & Gynecology·T H Bui, D R Jones
Dec 16, 2003·Birth Defects Research. Part C, Embryo Today : Reviews·Te-Chao Fang, Richard Poulsom
Jul 12, 2005·Bone Marrow Transplantation·C DawsonA R Gennery
Mar 25, 2011·The New England Journal of Medicine·Ornella Parolini
Jun 10, 1998·Springer Seminars in Immunopathology·A FischerM Cavazzana-Calvo
Nov 25, 1998·British Journal of Haematology·C W TurnerW H Fleming
Oct 9, 2002·Clinical Obstetrics and Gynecology·Alan W Flake
Jul 5, 2005·Expert Opinion on Investigational Drugs·D R Jones
Jun 24, 2010·Prenatal Diagnosis·Mahesh ChoolaniNicholas M Fisk

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