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Fatal late-onset EBV-associated post transplant lymphoproliferative disease after umbilical cord blood transplantation due to persistent mixed chimerism and severe delay in T-cell recovery in a patient with Omenn's syndrome

Bone Marrow Transplantation volume 34, pages 283–284 (2004)Cite this article

Epstein–Barr virus-associated post transplant lymphoproliferative disease (PTLD) is a typical and commonly fatal complication after allogeneic stem cell transplantation (SCT)1 with an incidence between 1 and 2%, independent of stem cell source.2, 3, 4, 5 The occurrence is highest in the first months after transplantation (ie early onset defined as PTLD <6 or 12 months after SCT) and only few cases present more than 1 year after SCT.4, 5 Here, we report about the, to our knowledge, first report of late-onset EBV-associated PTLD in a patient with Omenn's syndrome or SCID after umbilical cord blood transplantation.

An 18-month-old boy received unrelated umbilical cord blood transplantation with no HLA mismatch because of Omenn's syndrome, as reported previously.6 The umbilical cord blood was seronegative for EBV, and EBV PCR was negative as well. Conditioning regimen consisted of busulfan 5 mg/m2 p.o. once daily on days −10 through −7 (total dose 20 mg/m2), cyclophosphamide 50 mg/kg i.v. on days −5 to −2 (total dose 200 mg/kg) and antithymocyte globulin (ATG, rabbit Merieux) 5 mg/kg i.v. once daily on 4 consecutive days (days −5 to −2, total dose 20 mg/kg) according to the recommendations of the European Bone Marrow Transplantation (EBMT) working party for inborn errors. Numbers of infused cells were 7.2 × 107/kg body weight for nucleated and 0.5 × 105/kg for CD34+ cells. The patient received cyclosporin A and methylprednisolone for prevention of GvHD starting on day −1.

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Authors and Affiliations

  1. Universitätsklinik und Poliklinik für Kinder- und Jugendmedizin, Ernst-Grube Str., Halle, Germany

    A Meyer-Bahlburg, A Wawer, O Diwan, G Horneff & S Burdach

  2. Universitätsklinik und Poliklinik für Kinder- und Jugendmedizin Erlangen, Germany

    J Winkler

  3. Institut für Virologie und Antivirale Therapie der Friedrich-Schiller-Universität Jena, Germany

    A Meerbach & P Wutzler

  4. Institut für Pathologie der Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube Str., Halle, Germany

    H-J Holzhausen

  5. Department of Pediatrics, Technical University Munich, Koelnes Platz 1, Munich, D 80804, Germany

    A Wawer & S Burdach

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  1. A Meyer-Bahlburg
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  2. J Winkler
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  3. A Meerbach
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  8. G Horneff
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  9. S Burdach
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Meyer-Bahlburg, A., Winkler, J., Meerbach, A. et al. Fatal late-onset EBV-associated post transplant lymphoproliferative disease after umbilical cord blood transplantation due to persistent mixed chimerism and severe delay in T-cell recovery in a patient with Omenn's syndrome. Bone Marrow Transplant 34, 283–284 (2004). https://doi.org/10.1038/sj.bmt.1704564

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