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Ozsahin, Hülya; von Planta, Maya; Müller, Irene; Steinert, Hans C.; Nadal, David; Lauener, Roger; Tuchschmid, Peter; Willi, Ulrich V.; Ozsahin, Mahmut; Crompton, Nigel E.A.; Seger, Reinhard A.

Successful Treatment of Invasive Aspergillosis in Chronic Granulomatous Disease by Bone Marrow Transplantation, Granulocyte Colony-Stimulating Factor–Mobilized Granulocytes, and Liposomal Amphotericin-B

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  • Medientyp: E-Artikel
  • Titel: Successful Treatment of Invasive Aspergillosis in Chronic Granulomatous Disease by Bone Marrow Transplantation, Granulocyte Colony-Stimulating Factor–Mobilized Granulocytes, and Liposomal Amphotericin-B
  • Beteiligte: Ozsahin, Hülya; von Planta, Maya; Müller, Irene; Steinert, Hans C.; Nadal, David; Lauener, Roger; Tuchschmid, Peter; Willi, Ulrich V.; Ozsahin, Mahmut; Crompton, Nigel E.A.; Seger, Reinhard A.
  • Erschienen: American Society of Hematology, 1998
  • Erschienen in: Blood
  • Sprache: Englisch
  • DOI: 10.1182/blood.v92.8.2719
  • ISSN: 1528-0020; 0006-4971
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>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 γ-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.</jats:p><jats:p>© 1998 by The American Society of Hematology.</jats:p>
  • Zugangsstatus: Freier Zugang