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

Bone Marrow Transplantation volume 29, pages 625–628 (2002)Cite this article

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.

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Acknowledgements

The authors thank Dr W Böcher (Johannes Gutenberg University, Mainz, Germany) for kind support in the ELISPOT technique, Consuelo Pedras and Ana Hernández (laboratory technicians), Dr MA Llauradó for efficient administrative work regarding the BMT, and the patient's parents for their collaboration in this investigation.

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Authors and Affiliations

  1. Division of Immunology, Hospital General Universitario ‘Gregorio Marañón’, Madrid, Spain

    J Bartolomé, JJ Rodríguez-Molina, E Cela, A Cantalejo, E Fernández-Cruz & J Gil

  2. Division of Haematology, Hospital General Universitario ‘Gregorio Marañón’, Madrid, Spain

    A Gómez-Pineda

  3. Department of Pediatrics, Spedali Civili, Brescia, Italy

    F Porta, A Lafranchi, AG Ugazio & LD Notarangelo

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  1. J Bartolomé
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Bartolomé, J., Porta, F., Lafranchi, A. et al. B cell function after haploidentical in utero bone marrow transplantation in a patient with severe combined immunodeficiency. Bone Marrow Transplant 29, 625–628 (2002). https://doi.org/10.1038/sj.bmt.1703410

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