| Autor: |
Ozsahin, Hu¨lya, von Planta, Maya, Mu¨ller, Irene, Steinert, Hans C., Nadal, David, Lauener, Roger, Tuchschmid, Peter, Willi, Ulrich V., Ozsahin, Mahmut, Crompton, Nigel E.A., Seger, Reinhard A. |
| Zdroj: |
Blood; October 1998, Vol. 92 Issue: 8 p2719-2724, 6p |
| Abstrakt: |
X-linked chronic granulomatous disease (X-CGD) is a primary immunodeficiency with complete absence or malfunction of the nicotinamide adenine 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 contraindicated during invasive aspergillosis in any disease setting. We report an 8-year-old patient with CGD who underwent HLA-genoidentical BMT during invasive multifocal aspergillus nidulans infection, nonresponsive to treatment with amphotericin-B and ?-interferon. During the first 10 days post-BMT, the patient received granulocyte colony-stimulating factor (G-CSF)–mobilized, 25 Gy irradiated granulocytes from healthy volunteers plus G-CSF beginning on day 3 to prolong the viability of the transfused granulocytes. This was confirmed in vitro by apoptosis assays and in vivo by finding nitroblue tetrazolium (NBT)-positive granulocytes in peripheral blood 12 and 36 hours after the transfusions. Clinical and biological signs of infection began to disappear on day 7 post-BMT. Positron emission tomography with F18-fluorodeoxyglucose (FDG-PET) and computed tomography (CT) scans at 3 months post-BMT showed complete disappearance of infectious foci. At 2 years post-BMT, 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, provided that supportive therapy is optimal. © 1998 by The American Society of Hematology. |
| Databáze: |
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