Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Positive selection of high-affinity germinal center (GC) B cells is driven by antigen internalization through their B cell receptor (BCR) and presentation to follicular helper T cells. However, the requirements of BCR signaling in GC B cells remain poorly understood. Store-operated Ca2+ entry, mediated by stromal interacting molecule 1 (STIM1) and STIM2, is the main Ca2+ influx pathway triggered by BCR engagement. Here, we showed that STIM-deficient B cells have reduced B cell competitiveness compared with wild-type B cells during GC responses. B cell–specific deletion of STIM proteins decreased the number of high-affinity B cells in the late phase of GC formation. STIM deficiency did not affect GC B cell proliferation and antigen presentation but led to the enhancement of apoptosis due to the impaired upregulation of anti-apoptotic Bcl2a1. STIM-mediated activation of NFAT was required for the expression of Bcl2a1 after BCR stimulation. These findings suggest that STIM-mediated survival signals after antigen capture regulate the optimal selection and maintenance of GC B cells.

DOI： 10.1084/jem.20222178

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Publisher：金原出版  

DOI： 10.18888/sh.0000003140

CiNii Research

Publisher：診断と治療社  

DOI： 10.34433/pp.0000001146

CiNii Research

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Schizophyllum commune is a widely distributed basidiomycete fungus that occasionally causes sinusitis or allergic bronchopulmonary mycosis. The invasive infection mostly occurs in immunocompromised adults. The number of reports on S. commune infection have increased in this decade due to the expansion of diagnostic techniques and awareness in clinical practice. However, S.commune infection in patients with primary immunodeficiencies has not been reported yet. Here, we described S. commune-abscesses developed in the brain and lung of a boy with chronic granulomatous disease (CGD) after allogenic hematopoietic cell transplantation (HCT). A 12-year-old CGD patient developed febrile neutropenia from day 4 after HCT, followed by chest pain on day 23. He had no obvious infection before HCT. Diagnostic imaging revealed disseminated lung and brain abscesses. He received administration of voriconazole, and his symptoms improved after engraftment. Chronic administration of voriconazole had also a favorable therapeutic response to brain lesion. A part of the fungus ball exhaled by the patient was cultured to develop a filamentous fungus. S. commune was identified by the analysis of the 28S rRNA gene. The catalase test was positive for S. commune, indicating that S. commune had virulence in this patient with CGD. The assessment of specific-IgG to S. commune suggested peri-transplant infection, although colonization was not excluded. This rare pediatric case of S. commune infection highlights that CGD patients are vulnerable to invasive infection, especially when undergoing HCT.

DOI： 10.1016/j.jiac.2022.10.015

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Language：English   Publisher：エルゼビア・ジャパン(株)  

症例は12歳男児で、慢性肉芽腫性疾患の治癒を目的とした臍帯血移植を受けるために当院に入院した。ブスルファンとフルダラビンの移植前処置後に臍帯血を用いた同種造血幹細胞移植を施行した。移植後4日目から発熱性好中球減少症を発症し、23日目に胸痛を発症した。移植前に明らかな感染症はなかった。画像診断で播種性肺膿瘍および脳膿瘍が認められた。ボリコナゾール投与を行い、生着後、症状は改善した。ボリコナゾールの慢性投与により、脳膿瘍の治療効果も良好であった。患者の吐き出した菌球の一部を培養したところ、糸状菌が発生した。28S rRNA遺伝子の解析によりSchizophyllum commune(スエヒロタケ)を同定した。カタラーゼテストはS.communeに陽性であり、S.communeが本症例に病原性を有することが示された。S.communeに対する特異的IgGの評価から、定着は否定されなかったが、移植での周術期感染が示唆された。

Event date： 2023.11

Language：Japanese  

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Event date： 2023.10

Language：Japanese  

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Event date： 2023.10

Language：Japanese  

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Event date： 2022.10

Language：Japanese  

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Language：Japanese  

Language：Japanese   Publisher：(株)診断と治療社  

＜文献概要＞▽慢性活動性EBウイルス病(CAEBV)はその発症メカニズムがいまだ不明な難治性致死性疾患である.▽CAEBVは造血細胞移植を含む適切な治療によって,生命予後の改善が期待できる疾患である.▽現時点ではCAEBVに対する公費補助制度として小児慢性特定疾病のみ申請が可能だが,若年成人でも多く発症があり指定難病として認定されることが望ましい.

Language：Japanese   Publisher：(有)科学評論社