Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyria.

[erythropoietic protoporphyria]

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 .