TY - JOUR
T1 - B-cell recovery after stem cell transplantation of Artemis-deficient SCID requires elimination of autologous bone marrow precursor-B-cells
AU - Van Der Burg, Mirjam
AU - Weemaes, Corry M.R.
AU - Preijers, Frank
AU - Brons, Paul
AU - Barendregt, Barbara H.
AU - Van Tol, Maarten J.D.
AU - Hoogerbrugge, Peter
AU - Van Dongen, Jacques J.M.
PY - 2006/12
Y1 - 2006/12
N2 - 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.
AB - 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.
KW - Artemis-deficient SCID
KW - B-cell recovery
KW - Stem cell transplantation
UR - http://www.scopus.com/inward/record.url?scp=33845954952&partnerID=8YFLogxK
M3 - Article
C2 - 17145611
AN - SCOPUS:33845954952
SN - 0390-6078
VL - 91
SP - 1705
EP - 1709
JO - Haematologica
JF - Haematologica
IS - 12
ER -