Scientific article
Case report
English

Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin-B

Published inBlood, vol. 92, no. 8, p. 2719-2724
Publication date1998
Abstract

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 gamma-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.

Keywords
  • Amphotericin B/administration & dosage/therapeutic use
  • Antifungal Agents/administration & dosage/therapeutic use
  • Apoptosis
  • Aspergillosis/drug therapy/prevention & control/radionuclide imaging/therapy
  • Aspergillus nidulans
  • Bone Marrow Transplantation
  • Child
  • Combined Modality Therapy
  • Drug Carriers
  • Graft Survival/drug effects
  • Granulocyte Colony-Stimulating Factor/therapeutic use
  • Granulocytes/physiology
  • Granulomatous Disease, Chronic/complications/therapy
  • Humans
  • Itraconazole/therapeutic use
  • Leukocyte Count
  • Leukocyte Transfusion
  • Liposomes
  • Lung Diseases, Fungal/drug therapy
  • Male
  • Tomography, Emission-Computed
  • Treatment Outcome
Citation (ISO format)
OZSAHIN, Ayse Hulya et al. Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin-B. In: Blood, 1998, vol. 92, n° 8, p. 2719–2724.
Main files (1)
Article (Published version)
accessLevelRestricted
Identifiers
ISSN of the journal0006-4971
453views
0downloads

Technical informations

Creation07/23/2015 11:39:00 AM
First validation07/23/2015 11:39:00 AM
Update time03/14/2023 11:32:18 PM
Status update03/14/2023 11:32:17 PM
Last indexation10/31/2024 1:01:14 AM
All rights reserved by Archive ouverte UNIGE and the University of GenevaunigeBlack