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Sequential renal and bone marrow transplants in a child with Fanconi anemia.

Authors
  • Vincent, Carol L1, 2
  • Primack, William A1, 2, 3
  • Hipps, John1, 4
  • Kasow, Kimberly A1, 4
  • 1 Department of Pediatrics, University of North Carolina, Chapel Hill, NC, USA.
  • 2 Department of Medicine, University of North Carolina, Chapel Hill, NC, USA.
  • 3 Division of Nephrology, University of North Carolina, Chapel Hill, NC, USA.
  • 4 Division of Pediatric Hematology/Oncology, University of North Carolina, Chapel Hill, NC, USA.
Type
Published Article
Journal
Pediatric Transplantation
Publisher
Wiley (Blackwell Publishing)
Publication Date
February 2016
Volume
20
Issue
1
Pages
146–150
Identifiers
DOI: 10.1111/petr.12619
PMID: 26481770
Source
Medline
Keywords
License
Unknown

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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