B-cell recovery after stem cell transplantation of Artemis-deficient SCID requires elimination of autologous bone marrow precursor-B-cells.
Severe combined immunodeficiencies (SCID) are commonly fatal early in life. Adequate diagnosis and rapid institution of treatment, such as allogeneic stem cell transplantation (SCT), is essential. Several studies demonstrated that reconstitution of B-cell function after SCT is better in B-positive SCID than in B-negative SCID. We demonstrate that B-cell reconstitution in a B-negative SCID patient due to an Artemis mutation required the elimination of the autologous precursor-B-cells in bone marrow, probably to create physical space in the precursor-B-cell niches. Apparently, occupation of the precursor-B-cell niches is a potential dominant factor influencing repopulation of a functional B-cell compartment in B-negative SCID.
van der Burg
Mirjam
M
Department of Immunology, Erasmus MC, Dr. Molewaterplein 50, 3015 GE Rotterdam, The Netherlands.
Weemaes
Corry M R
CM
Preijers
Frank
F
Brons
Paul
P
Barendregt
Barbara H
BH
van Tol
Maarten J D
MJ
Hoogerbrugge
Peter
P
van Dongen
Jacques J M
JJ
D016175:B-Lymphocyte Subsets / Q000276:immunology*; D001854:Bone Marrow Cells / Q000276:immunology; D016026:Bone Marrow Transplantation / Q000379:methods; D004268:DNA-Binding Proteins; D004720:Endonucleases; D005260:Female; D006412:Hematopoietic Stem Cells / Q000276:immunology; D006801:Humans; D007223:Infant; D008212:Lymphocyte Depletion; D009687:Nuclear Proteins / Q000172:deficiency* / Q000235:genetics*; D016511:Severe Combined Immunodeficiency / Q000235:genetics / Q000276:immunology* / Q000601:surgery; D033581:Stem Cell Transplantation / Q000379:methods; D019172:Transplantation Conditioning / Q000379:methods; D014184:Transplantation, Homologous / Q000276:immunology
D004268:DNA-Binding Proteins; D009687:Nuclear Proteins; C426344:DCLRE1C protein, human; D004720:Endonucleases