SUCCESSFUL TREATMENT OF INVASIVE ASPERGILLOSIS IN CHRONIC GRANULOMATOUS-DISEASE BY BONE-MARROW TRANSPLANTATION, GRANULOCYTE-COLONY-STIMULATING FACTOR-MOBILIZED GRANULOCYTES, AND LIPOSOMAL AMPHOTERICIN-B

X-linked chronic granulomatous disease (X-CGD) is a primary immunodefi ciency with complete absence or malfunction of the nicotinamide adenin e dinucleotide phosphate (NADPH) oxidase in the phagocytic cells. Life -threatening infections especially with aspergillus are common despite optimal antimicrobial therapy. Bone marrow transplantation (BMT) is c ontraindicated during invasive aspergillosis in any disease setting. W e report an 8-year-old patient with CGD who underwent HLA-genoidentica l BMT during invasive multifocal aspergillus nidulans infection, nonre sponsive to treatment with amphotericin-B and gamma-interferon. During the first 10 days post-BMT, the patient received granulocyte colony-s timulating factor (G-CSF)-mobilized, 25 Gy irradiated granulocytes fro m healthy volunteers plus G-CSF beginning on day 3 to prolong the viab ility of the transfused granulocytes, This was confirmed in vitro by a poptosis assays and in vivo by finding nitroblue tetrazolium (NBT)-pos itive granulocytes in peripheral blood 12 and 36 hours after the trans fusions. Clinical and biological signs of infection began to disappear on day 7 post-BMT, Positron emission tomography with F18-fluorodeoxyg lucose (FDG-PET) and computed tomography (CT) scans at 3 months post-B MT showed complete disappearance of infectious foci. At 2 years post-B MT, the patient is well with full immune reconstitution and no sign of aspergillus infection. Our results show that HLA-identical BMT may be successful during invasive, noncontrollable aspergillus infection, pr ovided that supportive therapy is optimal. (C) 1998 by The American So ciety of Hematology.