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Erschienen in: Journal of Clinical Immunology 1/2017

06.12.2016 | Original Article

Resolution of Multifocal Epstein-Barr Virus-Related Smooth Muscle Tumor in a Patient with GATA2 Deficiency Following Hematopoietic Stem Cell Transplantation

verfasst von: Mark Parta, Jennifer Cuellar-Rodriguez, Alexandra F. Freeman, Juan Gea-Banacloche, Steven M. Holland, Dennis D. Hickstein

Erschienen in: Journal of Clinical Immunology | Ausgabe 1/2017

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Abstract

We performed allogeneic hematopoietic stem cell transplantation in a patient with GATA2 deficiency and an Epstein-Barr virus (EBV)-related spindle cell tumor involving the liver and possibly bone. He received a matched-related donor transplant with donor peripheral blood stem cells following a myeloablative conditioning regimen. He achieved rapid and high levels of donor engraftment and had complete reversal of the clinical and immunologic phenotype of MonoMAC/GATA2 deficiency and eradication of the EBV tumors after 3 years of follow-up. Thus, allogeneic hematopoietic stem cell transplant results in reconstitution of immunologic function and cure of EBV-associated malignancy in MonoMAC/GATA2 deficiency.
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Metadaten
Titel
Resolution of Multifocal Epstein-Barr Virus-Related Smooth Muscle Tumor in a Patient with GATA2 Deficiency Following Hematopoietic Stem Cell Transplantation
verfasst von
Mark Parta
Jennifer Cuellar-Rodriguez
Alexandra F. Freeman
Juan Gea-Banacloche
Steven M. Holland
Dennis D. Hickstein
Publikationsdatum
06.12.2016
Verlag
Springer US
Erschienen in
Journal of Clinical Immunology / Ausgabe 1/2017
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-016-0360-8

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