記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Alveolar rhabdomyosarcoma (ARMS) shows a predilection for the peripheral extremities and is very rarely identified as a primary in the brain. Here, we report a case of ARMS with multiple lesions exclusively within the central nervous system (CNS). CASE DESCRIPTION: A 20-year-old man presented to our hospital with a gradually increasing headache and disturbance of consciousness. Neuroimaging showed hydrocephalus and multiple tumor lesions, including in the brainstem and cerebellum, with uniform gadolinium enhancement on T1-weighted magnetic resonance imaging, as well as spinal cord seeding. Cerebrospinal fluid (CSF) analysis showed a slightly elevated cell count (6/μL; normal, <5/μL) and highly elevated protein (153 mg/dL). In addition, atypical cells were cytologically identified in the CSF. No other laboratory findings were abnormal. Emergency ventricular drainage was performed to control cerebral pressure, followed by a biopsy to confirm the diagnosis. Histological examination revealed a fascicular arrangement of oval cells with eosinophilic cytoplasm and tumor cells with pleomorphic nuclei and prominent nucleoli. Immunohistochemical studies showed negative results for glial fibrillary acidic protein and positive results for desmin and myogenin. In addition, molecular analysis revealed that this tumor had the H3F3A p.Lys28Met mutation and no paired box (PAX)3-forkhead box O1 (FOXO1) or PAX7-FOXO1 fusion genes. ARMS was, therefore, diagnosed. Chemotherapy and radiotherapy were subsequently initiated, but tumor growth could not be controlled, and the patient died 6 months after surgery. CONCLUSION: This report describes an extremely rare case of ARMS arising exclusively within the CNS.

DOI： 10.25259/SNI_299_2024

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語  

The most common organisms isolated from pediatric catheter-related bloodstream infections (CRBSIs) are Gram-positive cocci, such as coagulase-negative staphylococci and Staphylococcus aureus. There are few formal reports of Brevibacterium casei infection and even fewer reports of CRBSI due to this Gram-positive rod. Here we report the first case of CRBSI due to B. casei in an 8-year-old girl with acute myeloid leukemia in Japan. The isolate exhibited decreased susceptibility to ß-lactam antibiotics. Antimicrobial therapy with meropenem and vancomycin, in addition to the removal of central venous catheter line, consequently led to a significant clinical improvement of the patient's symptoms. A literature review found available clinical courses in 16 cases (4 pediatric cases including our case) of B. casei infection. Our case and those in literature suggested that B. casei infection often occurs in patients with indwelling central venous catheters; the literature review further suggested that removal of central venous catheters is required in most cases. Special attention should be paid to the detection of opportunistic infections due to Brevibacterium spp. in immunocompromized children who are using a central venous catheter.

DOI： 10.1155/2021/6691569

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.4103/jcrt.JCRT_449_19

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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記述言語：日本語   出版者・発行元：(一社)日本小児神経学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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DOI： 10.1002/gcc.22734

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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DOI： 10.1155/2019/7890673

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© The Author(s) 2018. We evaluated the efficacy of linezolid treatment in 6 children with health care–associated meningitis or ventriculitis (HCAMV) caused by gram-positive cocci. All children were diagnosed and treated at the Ehime University Hospital between January 2010 and December 2017. Of these, 5 were treated with linezolid as an empirical therapy. In these 5 patients, vancomycin was initially used but was changed to linezolid because of cerebrospinal fluid (CSF) culture positivity (n = 3) and a high minimum inhibitory concentration of vancomycin (n = 2). The most common HCAMV pathogens were methicillin-resistant coagulase-negative staphylococci (n = 3). In 3 patients, vancomycin concentration was low in CSF but reached the target concentration in serum, while linezolid concentration was high in both CSF and serum. HCAMV treatment using antimicrobial agents with poor CSF penetration may increase the likelihood of therapy failure. Linezolid is more susceptible as the first-line treatment for HCAMV compared with vancomycin.

DOI： 10.1177/0009922818803399

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

【緒言】肋骨骨髄炎は全骨髄炎の中で約1%と非常に稀であり、骨成長期に多く発症するとされている。【症例】生来健康な5歳男児。1ヵ月前から左側胸部痛が出現し、左第6肋骨に腫瘤を触知した。血液検査でWBC 7,700/μL(Neutro 58.5%)、CRP 3.87mg/dLと炎症反応の上昇を認めた。胸部CT検査で左第6肋骨に骨融解を伴う軟部腫瘤影、MRI T1強調画像でlow intensity、T2強調画像でhigh intensityを示す13mm×14mm×15mmの腫瘤を認めた。切開生検術を施行し、病理検査、細菌検査結果からStaphylococcus aureus(MSSA)による左第6肋骨骨髄炎と診断した。掻爬術と合計6週間の抗菌薬治療により治癒した。【結語】肋骨骨髄炎では、発熱といった全身症状がなく局所症状のみの症例も存在することから、小児の肋骨部腫瘤では鑑別すべき疾患の一つと考えられる。(著者抄録)

J-GLOBAL

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© 2018, © 2018 Taylor &amp; Francis Group, LLC. Although outcomes for infant leukemia have improved recently, transient adrenal insufficiency is commonly observed during treatment, especially after glucocorticoid administration. We identified three infants with acute leukemia who suffered from prolonged adrenal insufficiency requiring long-term (from 15 to 66 months) hydrocortisone replacement. All infants showed life-threatening symptoms associated with adrenal crisis after viral infections or other stress. Severe and prolonged damage of hypothalamo-pituitary-adrenal (HPA) axis is likely to occur in early infants with leukemia, therefore routine tolerance testing to evaluate HPA axis and hydrocortisone replacement therapy are recommended for infants with leukemia to avoid life-threatening complications caused by adrenal crisis.

DOI： 10.1080/08880018.2018.1539148

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DOI： 10.1111/ped.13540

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© 2018, © 2018 Taylor &amp; Francis Group, LLC. Primary bone marrow lymphoma (PBML) is hard to diagnose in children, due to the difficult identification of malignant cells in bone marrow. The first case, a 5-year-old boy, showed knee swelling with an intermittent fever. The second case, a 12-year-old girl, showed fever of unknown origin without lymphadenopathy or hepatosplenomegaly. In both cases, the diagnosis was not confirmed despite the repeated bone marrow aspirations. Finally, bone marrow aspiration and biopsy at the positive site by positron emission tomography (PET)-CT contributed to definitive diagnosis of PBML. The PET-CT is useful for the accurate diagnosis of PBML in children with non-specific symptoms.

DOI： 10.1080/08880018.2018.1459984

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記述言語：英語   出版者・発行元：WILEY-BLACKWELL  

DOI： 10.1002/pbc.26145

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© 2015 Japan Pediatric Society. Tsukamurella spp. infection is a rare but important cause of bacteremia in immunocompromised patients. The organism is an aerobic, Gram-positive, weakly acid-fast bacillus that is difficult to differentiate from other aerobic Actinomycetales by standard laboratory methods. Here, we report on the case of a 14-year-old patient with Hodgkin&#039;s lymphoma who, after intensive chemotherapy, developed Tsukamurella inchonensis bacteremia, which was identified on the peripherally inserted central venous catheter (PICC) using 16S rRNA sequencing analysis. The bacteremia was successfully controlled with antimicrobial therapy and subsequent removal of the PICC. This is the first report of bacteremia by Tsukamurella inchonensis in immunocompromised children. Careful observation and prompt analysis of opportunistic infection, including Tsukamurella spp., is very important in immunocompromised children.

DOI： 10.1111/ped.12472

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記述言語：日本語  

J-GLOBAL

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a subtype of acute myeloid leukemia, affecting mainly the elderly. It is thought to be derived from plasmacytoid dendritic cell precursors, which frequently present as cutaneous lesions. We have made a detailed analysis of an infant with BPDCN, who manifested with hemophagocytic lymphohistiocytosis. The peripheral blood leukocytes revealed the t(2;17;8)(p23;q23;p23) translocation and a CLTC-ALK fusion gene, which have never been reported in BPDCN or in any myeloid malignancies thus far. Neonatal blood spots on the patient's Guthrie card were analyzed for the presence of the CLTC-ALK fusion gene, identifying the in utero origin of the leukemic cell. Although the leukemic cells were positive for CD4, CD56, CD123, and CD303, indicating a plasmacytoid dendritic cell phenotype, detailed analysis of the lineage distribution of CLTC-ALK revealed that part of monocytes, neutrophils, and T cells possessed the fusion gene and were involved in the leukemic clone. These results indicated that leukemic cells with CLTC-ALK originated in a multipotent hematopoietic progenitor in utero. This is the first report of the CLTC-ALK fusion gene being associated with a myeloid malignancy, which may give us an important clue to the origin of this rare neoplasm. (c) 2013 Wiley Periodicals, Inc.

DOI： 10.1002/gcc.22119

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