SUCCESSFUL BONE-MARROW TRANSPLANTATION IN A CHILD WITH COMBINED IGG SUBCLASS DEFICIENCY AND NEUTROPENIA

An 8-year-old boy with combined IgG1 deficiency and neutropenia underw ent allogeneic BMT from his HLA-identical, MLC-negative sister, becaus e immunoglobulin (Ig) infusions and prophylactic antibiotics failed to prevent life-threatening infections. Conditioning was with busulfan a nd cyclophosphamide. MTX and CYA were given for the prophylaxis of GVH D. For 16 months after BMT no serious infections have occurred and ser um IgG1 levels have returned to the normal range without Ig replacemen t. BMT may be appropriate treatment for patients with IgG subclass def iciency who rarely respond to conservative therapy.