RESUMO
Cord blood transplantation (CBT) is curative for many primary immunodeficiencies (PIDs) but is associated with risks of viral infection and graft-versus-host disease (GvHD). Serotherapy reduces GvHD but potentially increases the risk of viral infection by delaying immune reconstitution. Because many PID patients have pre-existing viral infections, the optimal dose of serotherapy is unclear. We performed a retrospective analysis in 34 consecutive PID patients undergoing CBT and compared immune reconstitution, viral infection, GvHD, mortality, and long-term immune function between high-dose (n = 11) and low-dose (n = 9) serotherapy. Serotherapy dose had no effect on neutrophil engraftment. Median CD3(+) engraftment occurred at 92.5 and 97 days for high- and low-dose serotherapy, respectively. The low-dose serotherapy group had higher CD3(+), CD4(+), and early thymic emigrant counts at 4 months compared with the high-dose group. GvHD severity and number of viral infections did not differ between serotherapy doses. Survival from the transplantation process was 90.9% for high-dose and 100% for low-dose groups. In conclusion, low-dose serotherapy enhanced T cell reconstitution and thymopoiesis during the first year after CBT with no increase in GvHD.
Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical/efeitos adversos , Imunização Passiva/métodos , Transplante Homólogo/efeitos adversos , Adolescente , Adulto , Criança , Quimerismo , Feminino , Humanos , Síndromes de Imunodeficiência/etiologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
A female infant presented at birth with respiratory distress, which was subsequently shown to be secondary to lymphoid bronchiolitis, an exceptionally rare condition in childhood. Over the following 13 years there has been a slow progressive deterioration in her respiratory status with forced expiratory volume in 1 sec currently 40% predicted. Tests for connective tissue disease, infection, or immunodeficiency have all been negative and in the absence of any other explanation we postulate that this severe problem may have occurred as a consequence of an unrecognized intrauterine infection.
