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

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• 作者：Mark Parta ; Jennifer Cuellar-Rodriguez…
• 关键词：GATA2 ; Epstein ; Barr virus
• 刊名：Journal of Clinical Immunology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：37
• 期：1
• 页码：61-66
• 全文大小：
• 刊物类别：Biomedical and Life Sciences
• 刊物主题：Immunology; Infectious Diseases; Internal Medicine; Medical Microbiology;
• 出版者：Springer US
• ISSN：1573-2592
• 卷排序：37

文摘

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.