[Chediak-Higashi disease: a new case treated by bone marrow allograft]

We report a new case of Chediak-Higashi disease successfully treated by the transplantation of allogeneic bone marrow. Recurrent infections led to the diagnosis of the disease at the age of 15 months. At two and a half years of age, during a phase of accelerated disease activity, the patient received a bone marrow transplant donated by an HLA-identical brother. The patient was conditioned by chemotherapy alone; T-cells were removed from the graft and cyclosporin A was given to prevent graft-versus-host disease. Evidence of acceptance of the transplant was apparent 14 days after the procedure. Two months after the transplant, the blood count was normal, NK activity was satisfactory and no evidence of GVH disease was present. Incomplete hematopoietic chimerism was found (with two erythrocyte and lymphocyte populations). After four years follow-up, the patient is doing well and has no infections or evidence of active disease.