Resolution of Multifocal Epstein-Barr Virus-Related Smooth Muscle Tumor in a Patient with GATA2 Deficiency Following Hematopoietic Stem Cell Transplantation.
J Clin Immunol
; 37(1): 61-66, 2017 01.
Article
in En
| MEDLINE
| ID: mdl-27924436
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Smooth Muscle Tumor
/
Herpesvirus 4, Human
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Hematopoietic Stem Cell Transplantation
/
Epstein-Barr Virus Infections
/
GATA2 Transcription Factor
Limits:
Adult
/
Humans
/
Male
Language:
En
Journal:
J Clin Immunol
Year:
2017
Type:
Article
Affiliation country:
United States