B-cell recovery after stem cell transplantation of Artemis-deficient SCID requires elimination of autologous bone marrow precursor-B-cells

Mirjam Van Der Burg, Corry M.R. Weemaes, Frank Preijers, Paul Brons, Barbara H. Barendregt, Maarten J.D. Van Tol, Peter Hoogerbrugge, Jacques J.M. Van Dongen

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1705-1709
Number of pages5
JournalHaematologica
Volume91
Issue number12
Publication statusPublished - Dec 2006
Externally publishedYes

Keywords

  • Artemis-deficient SCID
  • B-cell recovery
  • Stem cell transplantation

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