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A 52-year-old man underwent non-myeloablative stem cell transplantation from his HLA identical sister for the treatment of mantle cell lymphoma. On day 0, he developed a high-grade fever, watery diarrhea and vesicles scattered on the skin. Well experienced dermatologists diagnosed these lesions as VZV reactivation. High dose antiviral agents were ineffective, and Fusarium solani was cultured from his stool and sputum. Systemic fusariosis progressed rapidly and he died of multiorgan failure on day 18. It is sometimes difficult to differentiate between viral and fungal blisters based on macroscopic examinations. We recommend early histopathological examination of the skin, when HSCT recipients develop vesicles. [ABSTRACT FROM AUTHOR]

Background: Extended-spectrum β-lactamase (ESBL)-producing Escherichia coli has been increasingly recognized as the cause of upper urinary tract infection (UTI) in children. We have been using flomoxef at our department since 2017 as the first-line empiric therapy for children diagnosed with UTIs, and we avoid using carbapenems, which are considered the first-line treatment for ESBL-producing E. coli . However, reports on the use of flomoxef for UTIs are limited, especially for pediatric patients. The presence of vesicoureteral reflux at the onset of pyelonephritis is a concern. Severe vesicoureteral reflux can lead to repeated UTI and future deterioration of renal function, but the indication for voiding urethrography, which closely examines the presence of vesicoureteral reflux complications, is controversial., Methods: We retrospectively reviewed the laboratory findings, treatment, and clinical course of 96 pyelonephritis cases experienced at our department over a 7-year period from April 2014 to March 2021., Results: ESBL-producing E. coli were identified as the cause of pyelonephritis in 51% of cases, and this value was significantly higher (88%) in 2017. No significant differences were found in the febrile period or recurrence rate between the flomoxef-initiated group and other antibiotics groups. We also examined clinical indicators to predict vesicoureteral reflux and found no significant differences in ultrasonographic findings of hydronephrosis., Conclusion: In the present series, 51% of all pyelonephritis cases were found to be caused by ESBL-producing E. coli , with a significant increase in recent years. Flomoxef may be a useful alternative to carbapenem for ESBL-producing E. coli and the initial antibiotic of choice for upper UTIs in children. The indication for voiding cystourethrography should be carefully determined., Competing Interests: The authors declare no conflict of interest., (©2023 Tottori University Medical Press.)

No standard treatment for relapsed or refractory anaplastic large-cell lymphoma (ALCL) has been established. This study is a multicenter, open-label trial to examine the effectiveness and safety of transplantation with reduced-intensity conditioning (RIC) for patients under 20 years old with relapsed or refractory ALCL. We defined RIC as the administration of fludarabine (30 mg/m2/day) for five days plus melphalan (70 mg/m2/day) for two days and total body irradiation at 4 Gy, followed by allogeneic hematopoietic stem cell transplantation., Competing Interests: No potential conflict of interest relevant to this article was reported.

This trial enrolls patients with untreated Hodgkin's lymphoma aged<20 years at diagnosis and examines the effects of omitting radiation therapy if the FDG-positron emission tomography (PET) findings after two completed cycles of combination chemotherapy are negative. It thereby aims to determine whether patients who truly require radiation therapy can be identified by FDG-PET. If so, this modality could be used to omit radiation therapy for all other patients, decreasing the risk of serious long-term complications without affecting survival rates. The outcomes of patients for whom FDG-PET is used to assess early treatment response will also be determined., Competing Interests: No potential conflict of interest relevant to this article was reported.

Neuroblastoma (NB) is a childhood solid malignant tumor originating from precursor cells of the peripheral nervous system. We have previously established a risk classification system based on DNA copy number profiles. To further explore the pathogenesis of NBs in distinct risk groups, we performed whole-exome sequencing analysis of 57 primary and 7 recurrent/metastatic tumors with unique chromosomal aberration profiles as categorized by our genomic sub-grouping system. Overall, a low frequency of somatic mutations was found. Besides ALK (4/64, 6.3%), SEMA6C , SLIT1 and NRAS , genes involved in the axon guidance pathway, were identified as recurrently mutated in 6 of 64 tumors (9.4%). Pathway enrichment analysis revealed enrichment of 25 mutated genes in the mitogen-activated protein kinase (MAPK) pathway, 13 genes in the Wnt pathway, and 12 genes in the axon guidance pathway. Genomic analyses demonstrated that primary and matched recurrent or metastatic tumors obtained from sporadic and monozygotic twin NBs were clonally related with variable extents of genetic heterogeneity. Monozygotic twin NBs displayed different evolutionary trajectories. These results indicate the involvement of the axon guidance, MAPK and Wnt pathways in NB and demonstrate genomic diversity with NB progression., Competing Interests: CONFLICTS OF INTEREST The authors have declared that no competing interests exist.

Mutations in RAS, neurofibromatosis type 1 (NF1), and PTPN11, constituents of the granulocyte-macrophage colony-stimulating factor signaling pathway, have been recognized in patients with juvenile myelomonocytic leukemia (JMML). We assessed 71 children with JMML for NRAS, KRAS, and PTPN11 mutations and evaluated their clinical significance. Of the 71 patients, three had been clinically diagnosed with neurofibromatosis type 1, and PTPN11 and NRAS/KRAS mutations were found in 32 (45%) and 13 (18%) patients, respectively. No simultaneous aberrations were found. Compared with patients with RAS mutation or without any aberrations, patients with PTPN11 mutation were significantly older at diagnosis and had higher fetal Hb levels, both of which have been recognized as poor prognostic factors. As was expected, overall survival was lower for patients with the PTPN11 mutation than for those without (25 versus 64%; p = 0.0029). In an analysis of 48 patients who received hematopoietic stem cell transplantation, PTPN11 mutations were also associated with poor prognosis for survival. Mutation in PTPN11 was the only unfavorable factor for relapse after hematopoietic stem cell transplantation (p = 0.001). All patients who died after relapse had PTPN11 mutation. These results suggest that JMML with PTPN11 mutation might be a distinct subgroup with specific clinical characteristics and poor outcome.

Invasive aspergillosis is a common form of fungal infection in patients with hematological malignancies. Because Aspergillus species have angioinvasive properties, they frequently disseminate from the lung to a variety of organs via hematogenous spread. Extra-pulmonary involvement occurs at an advanced stage of invasive aspergillosis, and represents an ominous sign. However, few reports have been published on extra-pulmonary involvement in cases of aspergillosis. Its clinical features have not been fully clarified. We experienced a patient who developed thyrotoxicosis and fatal airway obstruction caused by invasive aspergillosis of the thyroid. A 26-year-old man was admitted to our hospital for the treatment of non-Hodgkin's lymphoma. During myelosuppression following the chemotherapy, he developed cervical swelling and hyperthyroidism. We suspected lymphoma infiltration to the thyroid, and irradiated it with a total of 26 Gy. However, the cervical lesion enlarged rapidly, and he complained of wheezing and dyspnea. We underwent immediate tracheostomy to secure the airway, but he died. Autopsy findings were striking. Extensive necrosis with diffuse infiltration of Aspergillus hyphae was observed in the thyroid gland. Necrotic tissues of the thyroid protruded into the tracheal lumen, causing airway obstruction. This case demonstrated that invasive aspergillosis of the thyroid can lead to medical emergency.