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| Title: | B-cell recovery after stem cell transplantation of Artemis-deficient SCID requires elimination of autologous bone marrow precursor-B-cells. |
| Author(s): | Burg, M. van der Weemaes, C.M.R. (070242895) Preijers, F.W.M.B. (07401112X) Brons, P.P.T. (114596875) Barendregt, B.H. Tol, M. van Hoogerbrugge, P.M. (073498661) Dongen, J.J.M. van |
| Publication year: | 2006 |
| Document type: | Article / Letter to editor |
| Journal: | Haematologica |
| ISSN: | 0390-6078 |
| Volume: | vol. 91 |
| Issue: | iss. 12 |
| Start page: | p. 1705 |
| End page: | p. 1709 |
| Abstract: | 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. |
| Subject: | UMCN 1.2: Molecular diagnosis, prognosis and monitoring UMCN 1.4: Immunotherapy, gene therapy and transplantation |
| Organization: | UMCN Extern Paediatrics CHL |
| Appears in Collections: | Academic bibliography
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Please use this identifier to cite or link to this item:
http://hdl.handle.net/2066/50855
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