A 34-year-old man presented with fever 3 months after developing acute hepatitis of unknown etiology. Laboratory analyses revealed pancytopenia, and bone marrow biopsy confirmed the diagnosis of aplastic anemia (AA). He developed severe AA, becoming neutropenic, which led to invasive infections with liver abscesses that was possible to have been caused by Aspergillus species. Micafungin was not effective, and treatment with liposomal amphotericin B (L-AMB) and voriconazole (VRCZ) did not alter the size or activity of the lesion, but the patient’s body temperature returned to normal. He was referred to our hospital for emergent allogeneic hematopoietic stem cell transplantation (HSCT). However, a human leukocyte antigen (HLA)-identical donor was not found, and peripheral blood HSCT using a reduced-intensity conditioning regimen with post-transplant high-dose cyclophosphamide from an HLA-haploidentical sibling donor was considered. L-AMB was discontinued before the conditioning for HSCT, but VRCZ was continued. Neutrophil engraftment was obtained after 13 days from HSCT. No adverse events due to the graft versus host disease or recurrence of liver abscess occurred. Haploidentical HSCT using peripheral blood stem cells mobilized by granulocyte colony-stimulating factor may be a treatment option for severe AA patients with invasive fungal infections if an HLA-matching donor cannot be found.
A 17-year-old male with chronic granulomatous disease received allogeneic hematopoietic stem cell transplantation (HSCT) from an HLA-matched unrelated donor. He was taking prednisolone because of acute graft-versus-host disease that developed 5 months after HSCT, and bronchiolitis obliterans was diagnosed 9 months after HSCT. His respiratory symptoms improved after increasing the prednisolone dose; however, a nodule with a diameter of 3 cm was detected in the right upper lobe on CT scan 12 months after HSCT. Serological markers for fungi were negative and bronchoalveolar lavage (BAL) was performed. Nocardia abscessus was detected from the BAL fluid, establishing the diagnosis of pulmonary nocardiosis. A therapeutic dose of sulfamethoxazole-trimethoprim was effective with an improvement in pulmonary nodules. Nocardia is relatively slow-growing and difficult to culture. The differentiation of nocardiosis from mycobacterial and fungal infections by imaging is also difficult. The differential diagnosis of respiratory infections in HSCT patients is broad, yet microbiological diagnosis is often delayed due to avoidance of invasive procedures in children. BAL is beneficial for early diagnosis and averting unnecessary antifungal therapy.