• Tomonari Shigemura, Yozo Nakazawa, Kentaro Yoshikawa, Koichi Hirabayashi, Shoji Saito, Norimoto Kobayashi, Kazuo Sakashita, Masaaki Shiohara, Taizo Wada, Shigetaka Shimodaira, Kazunaga Agematsu, and Kenichi Koike.
• Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan; Division of Hematology, Oncology, and Immunology, Nagano Children's Hospital, Azumino, Japan; Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan; Division of Transfusion Medicine, Cell Processing Center, Shinshu University Hospital; Department of Infection and Host Defense, Shinshu University, Graduate School of Medicine, Matsumoto, Japan.
• Transfusion. 2014 Mar 1; 54 (3): 516-21.

BackgroundChronic granulomatous disease (CGD) is a rare inherited primary immunodeficiency that affects phagocytic cells. CGD patients are susceptible to fungal infections, especially Aspergillus infections. The management of life-threatening Aspergillus infections in CGD is particularly difficult because some infections cannot be eradicated with standard antifungal treatments and, hence, result in death.Case ReportA 2-week-old girl developed invasive pulmonary aspergillosis, which rapidly progressed to respiratory failure. Liposomal amphotericin B, micafungin, and voriconazole were not effective. At the age of 2 months, she was diagnosed with p67phox-deficient CGD. In addition to antifungal treatment, the patient received 21 granulocyte transfusions (GTX), which were obtained from 300- or 400-mL whole blood samples from healthy random donors who were not treated with granulocyte-colony-stimulating factor or dexamethasone. The median neutrophil count of the GTX was 1.88 × 10(8) /kg body weight. Rituximab was administered to reduce alloimmunization to human leukocyte antigens (HLA) after the eighth GTX, resulting in their absence of anti-HLA before and after cord blood transplantation (CBT). A marked improvement in her invasive pulmonary aspergillosis was achieved, although the first CBT was rejected. Complete hematopoietic recovery was obtained after the second CBT.ConclusionRepeated GTX containing relatively low doses of neutrophils might be able to control severe Aspergillus infections in infants with CGD.© 2013 American Association of Blood Banks.

21 keywords...

hide…