title: Successful treatment of invasive aspergillosis in chronic granulomatous disease by granulocyte transfusions followed by peripheral blood stem cell transplantation.
abstract: Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder characterized by impaired microbial killing and susceptibility to bacterial and fungal infections. Cure of the disease can be achieved by stem cell transplantation when performed early in its course, and before severe infections have developed. Invasive aspergillosis constitutes a very high risk for transplantation. We report a 4-year-old boy with X-linked CGD who underwent successful HLA-identical peripheral blood stem cell (PBSC) transplantation during invasive pulmonary aspergillosis and osteomyelitis of the left fourth rib, which was unresponsive to antifungal treatment. During the 2 months prior to the transplant he received G-CSF-mobilized granulocyte transfusions (GTX) from unrelated donors three times a week in addition to the antifungal treatment. This resulted in clinical improvement in his respiratory status. He also received GTX during the aplastic period after the conditioning regimen, until he had engrafted. Post-transplant superoxide generation test revealed that neutrophil function was within normal range. One year post transplant the CT scan showed almost complete clearance of the pulmonary infiltrates and a marked improvement in the osteomyelitic process. Based on other reports and our own experience, GTX can serve as important treatment in patients with CGD who have failed conventional anti-fungal treatment and for whom stem cell transplantation is the only chance for cure.
authors: Department of Pediatric Hematology/Oncology, Sheba Medical Center, Tel-Hashomer, Israel.
authors: B
authors: B
authors: Bielorai
authors: A
authors: A
authors: Toren
authors: B
authors: B
authors: Wolach
authors: M
authors: M
authors: Mandel
authors: H
authors: H
authors: Golan
authors: Y
authors: Y
authors: Neumann
authors: C
authors: C
authors: Kaplinisky
authors: M
authors: M
authors: Weintraub
authors: N
authors: N
authors: Keller
authors: N
authors: N
authors: Amariglio
authors: J
authors: J
authors: Paswell
authors: G
authors: G
authors: Rechavi
mesh_terms: D000666:Amphotericin B / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001228:Aspergillosis / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004352:Drug Resistance, Microbial; D005437:Flucytosine / Q000627:therapeutic use; D016179:Granulocyte Colony-Stimulating Factor / Q000494:pharmacology; D006098:Granulocytes / Q000637:transplantation*; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016867:Immunocompromised Host; D017964:Itraconazole / Q000627:therapeutic use; D017708:Leukocyte Transfusion; D008172:Lung Diseases, Fungal / Q000188:drug therapy / Q000209:etiology / Q000382:microbiology / Q000628:therapy*; D008297:Male; D009504:Neutrophils / Q000502:physiology; D010019:Osteomyelitis / Q000188:drug therapy / Q000209:etiology / Q000382:microbiology / Q000628:therapy*; D011743:Pyrimidines / Q000627:therapeutic use; D016897:Respiratory Burst; D012272:Ribs / Q000382:microbiology; D014230:Triazoles / Q000627:therapeutic use; D065819:Voriconazole
chemical_list: D000935:Antifungal Agents; D011743:Pyrimidines; D014230:Triazoles; D016179:Granulocyte Colony-Stimulating Factor; D017964:Itraconazole; D000666:Amphotericin B; D005437:Flucytosine; D065819:Voriconazole