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- Title
Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyria.
- Authors
Smiers, Frans J.; Van de Vijver, Els; Delsing, Bas J. P.; Lankester, Arjan C.; Ball, Lynne M.; Rings, Edmund H. H. M.; van Rheenen, Patrick F.; Bredius, Robbert G. M.
- Abstract
Smiers FJ, Van de Vijver E, Delsing BJP, Lankester AC, Ball LM, Rings EHHM, van Rheenen PF, Bredius RGM. Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyria. Pediatr Transplantation 2010: 14:471–475. © 2009 John Wiley & Sons A/S. A nine-yr-old boy with EPP suffered from severe skin burns and liver failure caused by progressive cholestasis and fibrosis. OLT was performed without major complications. Four months following liver transplantation he underwent parental haploidentical HSCT. The myeloablative conditioning regimen was relatively well tolerated and hematological engraftment was rapid (on day 10). Protoporphyrin concentrations returned to normal following HSCT. However, immune recovery was significantly delayed. Varicella zoster virus reactivation resulted in impaired vision, prolonged hospitalization and eventually in multiorgan failure and death. Sequential liver and haploidentical HSCT proved feasible though a high risk procedure in this EPP patient. The management of post-IST after these combined transplantations remains a challenge and needs to be further established.
- Subjects
LIVER transplantation; STEM cell transplantation; STEM cells; LIVER diseases; CHOLESTASIS
- Publication
Pediatric Transplantation, 2010, Vol 14, Issue 4, p471
- ISSN
1397-3142
- Publication type
Article
- DOI
10.1111/j.1399-3046.2009.01233.x