RESUMO
A girl with sickle cell anemia was treated with cord blood transplantation combined with hematopoietic growth factor. Cord blood cells were collected from a sister with an identical human leukocyte antigen complex who was a carrier of the sickle cell trait (hemoglobin AS). The patient had complete engraftment and no graft-versus-host disease. The persistence of a high level of fetal hemoglobin 6 months after engraftment was noted.
Assuntos
Anemia Falciforme/cirurgia , Sangue Fetal , Hemoglobina Fetal/metabolismo , Transplante de Medula Óssea , Pré-Escolar , Eletroforese , Feminino , Antígenos HLA , HumanosRESUMO
Two unrelated children with a severe form of juvenile hyaline fibromatosis are described. In addition to painful flexion contractures of all of the large joints, oral and skin lesions, and typical radiologic appearance of osteolytic defects, both girls had marked growth retardation and recurrent infections. Both children died in early infancy of overwhelming infection.