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

  1. Author:
    Parta, Mark [ORCID]
    Cuellar-Rodriguez, Jennifer
    Freeman, Alexandra F
    Gea-Banacloche, Juan
    Holland, Steven M
    Hickstein, Dennis D

  2. Author Address

    Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc., NCI Campus at Frederick, Frederick, MD, 21702, USA. mark.parta@nih.gov., Department of Infectious Diseases, Instituto Nacional de Ciencias M 233;dicas y Nutrici 243;n Salvador Zubiran, Mexico City, Mexico., Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Experimental Transplantation and Immunology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.,
    1. Year: 2017
    2. Date: Jan
    3. Epub Date: 2016 Dec 06
  2. Journal: Journal of clinical immunology
    1. 37
    2. 1
    3. Pages: 61-66
  4. Type of Article: Article
  1. 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 3 160;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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External Sources

  1. View Full Text
  2. DOI: 10.1007/s10875-016-0360-8
  3. PMID: 27924436
  4. View record in Web of Science®
  5. WOS: 000392298100015

Library Notes

  1. Fiscal Year: FY2016-2017
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