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1689. Active, Invasive Fungal Infection, and Hematopoietic Cell Transplantation (HCT) in Chronic Granulomatous Disease (CGD): Presentation, Procedure, and Outcomes

BACKGROUND: Individuals with CGD develop invasive fungal infections which are often refractory to medical therapy. Disease caused by Aspergillus nidulans (An), Scedosporium apiospermum (Sa), and Aspergillus viridinutans (Av), can be fatal due to involvement of structures contiguous to the primary pu...

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Detalles Bibliográficos
Autores principales: Parta, Mark, Zerbe, Christa, Holland, Steven M, Malech, Harry, Kang, Elizabeth M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6808658/
http://dx.doi.org/10.1093/ofid/ofz360.1553
Descripción
Sumario:BACKGROUND: Individuals with CGD develop invasive fungal infections which are often refractory to medical therapy. Disease caused by Aspergillus nidulans (An), Scedosporium apiospermum (Sa), and Aspergillus viridinutans (Av), can be fatal due to involvement of structures contiguous to the primary pulmonary infection. Protocols for managing an active fungal infection during HCT are lacking. Here we describe 5 patients transplanted with active fungal infection and the treatments ancillary to HCT that led to successful outcomes. METHODS: Four males and one female underwent peripheral blood HCT utilizing 3 different transplant platforms. Granulocyte transfusions (GCT) were used in 4 cases. RESULTS: Antifungal treatment and transplant outcomes are reported in Table 1. Patients 1 (Figure 1) and 4 (previously reported) had Sa infection of the lung and spine and lung and pericardium, respectively. Patients 2 (Figure 2) and 5 had An pneumonia with spinal and sternal involvement, respectively. Patient 3 had Av pneumonia involving the lower lung and diaphragm (Figure 3). The transplant protocol was modified to include a high stem cell dose to facilitate engraftment and post-transplant cyclophosphamide GVHD prophylaxis. No GCTs were used proximal to or during HCT due to the development of antineutrophil antibodies. CONCLUSION: Active fungal infection can be managed during HCT. Combination therapy (GCTs and antifungals) lessened the burden of disease prior to and during HCT. All patients had clinical resolution of their infection post-HCT and successful engraftment. Bone disease stabilized post-HCT and remineralization occurred. One patient was successfully transplanted without GCT and its potential complications. The authors have no conflicts to report. [Image: see text] [Image: see text] [Image: see text] [Image: see text] DISCLOSURES: All authors: No reported disclosures.