Article ; Online: Sequential renal and bone marrow transplants in a child with Fanconi anemia.
2016 Volume 20, Issue 1, Page(s) 146–150
Abstract: FA is an autosomal recessive disorder characterized by small stature and renal abnormalities. FA can lead to progressive bone marrow failure, myelodysplastic syndrome, or acute leukemia. Using a multidisciplinary team approach, we managed a 3-yr-old boy ... ...
| Abstract | FA is an autosomal recessive disorder characterized by small stature and renal abnormalities. FA can lead to progressive bone marrow failure, myelodysplastic syndrome, or acute leukemia. Using a multidisciplinary team approach, we managed a 3-yr-old boy with FA who simultaneously developed renal and hematopoietic failure. Because renal function was insufficient to support the conditioning regimen for HCT, we performed a deceased donor renal transplant in December 2012 prior to HCT with the known risk of graft-versus-graft rejection of the donor kidney. Seven months later he underwent allogeneic HCT. He obtained myeloid engraftment on day +11 and peripheral blood chimerism demonstrated all donor by day +21. He developed asymptomatic CMV reactivation and despite antirejection medications, mild skin graft-versus-host disease. He has maintained excellent renal function and remains transfusion independent with full hematopoietic recovery. He has not experienced any renal rejection episodes nor developed donor-specific antibodies toward his renal donor. Peripheral blood chimerism remains completely HCT donor. He is clinically well, now greater than two and a half yr after renal transplant and two yr after HCT. The continuing close collaboration between the Pediatric Nephrology and Bone Marrow Transplant teams is a major factor in this successful outcome. |
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| MeSH term(s) | Bone Marrow Transplantation ; Child, Preschool ; Fanconi Anemia/complications ; Fanconi Anemia/surgery ; Graft Rejection ; Graft Survival/immunology ; Hematologic Diseases/complications ; Hematologic Diseases/surgery ; Hematopoietic Stem Cell Transplantation ; Humans ; Interdisciplinary Communication ; Kidney Transplantation ; Male ; Renal Insufficiency/complications ; Renal Insufficiency/surgery ; Transplantation Conditioning ; Transplantation, Homologous | |||||
| Language | English | |||||
| Publishing date | 2016-02 | |||||
| Publishing country | Denmark | |||||
| Document type | Case Reports ; Journal Article | |||||
| ZDB-ID | 1390284-2 | |||||
| ISSN | 1399-3046 ; 1397-3142 | |||||
| ISSN (online) | 1399-3046 | |||||
| ISSN | 1397-3142 | |||||
| DOI | 10.1111/petr.12619 | |||||
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| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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