title: Beneficial effect of pre-transplant splenectomy on displacement bone marrow transplantation for Gaucher's syndrome.
abstract: 2 patients with fast and 4 with medium varieties of Gaucher's syndrome were treated by displacement bone marrow transplantation to install a donor enzyme factory for life. Immunoprophylaxis was given to prevent host impairment of normal enzymes. 5 patients showed rapid and remarkable improvement and were leading fully active lives at 310-1207 days postgraft. Hypersplenism caused the death of 1 patient and a stormy time for another. Pre-transplant elective splenectomy hastened recovery.
mesh_terms: D000293:Adolescent; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D005776:Gaucher Disease / Q000628:therapy*; D006801:Humans; D008297:Male; D008722:Methods; D013156:Splenectomy