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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. ; Preijers, F.W.M.B. ; Brons, P.P.T. ; Barendregt, B.H.; Tol, M. van; Hoogerbrugge, P.M. ; Dongen, J.J.M. van
Publication year: 2006
Source: Haematologica, vol. 91, iss. 12, (2006), pp. 1705-1709
ISSN: 0390-6078
Publication type: Article / Letter to editor

Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/50855   https://hdl.handle.net/2066/50855

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Subject: N4i 1: Pathogenesis and modulation of inflammation
NCMLS 2: Immune Regulation
ONCOL 1: Hereditary cancer and cancer-related syndromes
ONCOL 2: Age-related aspects of cancer
ONCOL 3: Translational research
UMCN 1.2: Molecular diagnosis, prognosis and monitoring
UMCN 1.4: Immunotherapy, gene therapy and transplantation
UMCN 1.4: Immunotherapy, gene therapy and transplantation
Organization: Paediatrics
CHL
Journal title:
Haematologica
Volume: vol. 91
Issue: iss. 12
Page start: p. 1705
Page end: 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.

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