T cell reconstitution by haploidentical BMT does not restore the diversification of the Ig heavy chain gene in patients with X-linked SCID

Y. Minegishi, N. Ishii, M. Tsuchida, H. Okawa, K. Sugamura, J. Yata

Research output: Contribution to journalArticlepeer-review

8 Citations (Scopus)

Abstract

We previously examined the Ig heavy (H) chain gene of pretransplant patients with X-linked SCID (XSCID), having defects in the gene of the IL-2 receptor (R)γ chain. In the present study, we analyzed two post-transplant XSCID patients, in whom T cell depleted haploidentical BMT resulted in lymphoid split chimeras, ie, donor functional T cells coexisting with recipient B cells. Although the recipient B cells produced IgM, no isohemagglutinin or Ag-specific Ab was detected. To investigate the cause of failure to produce Ab in the patients, we sequenced the complementarity determining region 3 (CDR3) and adjacent region of Ig H chain gene, which govern Ab specificity. Among the 64 post-transplant CDR3 junctional sequences, combinatorial and junctional diversity were normal compared with those in age-matched controls. All of the post-transplant joining regions except one clone were equal to germline and the frequency of somatic mutation was significantly lower than that in age-matched controls. The results indicated that T cell reconstitution by BMT does not restore diversification of the Ig gene in the IL-2Rγ chain-deficient B cells, which might be associated with the defect in the Ag-specific Ab production.

Original languageEnglish
Pages (from-to)801-806
Number of pages6
JournalBone Marrow Transplantation
Volume16
Issue number6
Publication statusPublished - 1995 Dec 1
Externally publishedYes

Keywords

  • Haploidentical BMT
  • Ig heavy chain
  • T cells
  • X-linked SCID

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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