B cell function after haploidentical in utero bone marrow transplantation in a patient with severe combined immunodeficiency

J. Bartolomé, F. Porta, A. Lafranchi, J. J. Rodríguez-Molina, E. Cela, A. Cantalejo, E. Fernández-Cruz, A. Gómez-Pineda, A. G. Ugazio, L. D. Notarangelo, J. Gil

Research output: Contribution to journalArticle

Abstract

An in utero paternal CD34+ cell transplant was performed in a T-B+NK+ SCID fetus. We report here the results of the 3-year humoral immune reconstitution study. The methods used were ApoB VNTR typing, flow cytometry, nephelometry, hemagglutination, ELISA, ELISPOT and lymphoproliferative assays. The T cells were of donor origin whereas monocytes, B and NK cells were of host origin. Peripheral B cell counts and IgM levels were normal since birth. IVIG therapy was required at 5 months of age until 2 years old. IgA levels ≥20 mg/dl were detected from month 17 post transplantation. Isohemagglutinins were present since month 8 post transplantation, the highest titers (anti-A:1/128, anti-B:1/32) were obtained at month 33 post-transplantation. After immunization with rHBsAg, circulating anti-HBsAg IgG secreting cells and a 7.8-fold increase in serum anti-HBsAg Ab were detected. We conclude that split chimerism following in utero haploidentical BMT allows complete humoral immune reconstitution in a T-B+NK+SCID patient.

Original languageEnglish
Pages (from-to)625-628
Number of pages4
JournalBone Marrow Transplantation
Volume29
Issue number7
DOIs
Publication statusPublished - 2002

Keywords

  • Bone marrow
  • Humoral
  • Immunodeficiency
  • In utero
  • Reconstitution
  • Stem cell transplantation

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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    Bartolomé, J., Porta, F., Lafranchi, A., Rodríguez-Molina, J. J., Cela, E., Cantalejo, A., Fernández-Cruz, E., Gómez-Pineda, A., Ugazio, A. G., Notarangelo, L. D., & Gil, J. (2002). B cell function after haploidentical in utero bone marrow transplantation in a patient with severe combined immunodeficiency. Bone Marrow Transplantation, 29(7), 625-628. https://doi.org/10.1038/sj/bmt/1703410