期刊
JOURNAL OF CLINICAL IMMUNOLOGY
卷 37, 期 1, 页码 61-66出版社
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-016-0360-8
关键词
GATA2; Epstein-Barr virus
类别
资金
- National Cancer Institute, National Institutes of Health [HHSN261200800001E]
- NIH, National Cancer Institute, Center for Cancer Research
- National Institute of Allergy and Infectious Diseases
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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