2012 Fiscal Year Final Research Report
Establishing rapid diagnostic method and unraveling the pathogenesis offamilial hemophagocytic lymphohistiocytosis
| Project/Area Number |
22591159
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Kyoto University |
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Principal Investigator |
YASUMI Takahiro 京都大学, 大学院・医学研究科, 講師 (00511891)
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| Co-Investigator(Kenkyū-buntansha) |
HEIKE Toshio 京都大学, 大学院・医学研究科, 教授 (90190173)
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| Research Collaborator |
NISHIKOMORI Ryuta 京都大学, 大学院・医学研究科, 准教授 (70359800)
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| Project Period (FY) |
2010 – 2012
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| Keywords | 原発性免疫不全症 / 家族性血球貪食性リンパ組織球症 / 迅速診断法 |
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| Research Abstract |
We established a new rapid screening test for FHL3/4/5 by detecting intra-platelet expression of munc13-4/syntaxin11/munc18-2 proteins and found that combinination ofCTL degranulation and munc13-4 expression assays is very useful for finding atypical, late-onset cases of FHL3. We also found that FHL can be clinically differentiated from certain types of secondary HLH cases by comparing levels of serum lactate dehydrogenase, sIL-2R, ferritin, and lymphocyte/neutrophil ratio in peripheral blood at early phase of the disease.
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[Journal Article] Robust and highly-efficientdifferentiation of functional monocytic cells from human pluripotent stem cells under serumand feeder cell-free conditions2013
Author(s)
Yanagimachi MD, Niwa A, Tanaka T, Honda-Ozaki F, Nishimoto S, Murata Y, Yasumi T, Ito J, Tomida S, Oshima K, Asaka I, Goto H, Heike T, Nakahata T, Saito MK
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Journal Title
PLoSOne
Volume: 8(4):e59243
DOI
Peer Reviewed
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[Journal Article] Reduced-intensity conditioning in unrelated donor cord blood transplantation for familial hemophagocytic lymphohistiocytosis2012
Author(s)
Nishi M, Nishimura R, Suzuki N, Sawada A, Okamura T, Fujita N, Kanai R, Yano J, Adachi S, Yasumi T, Sato E, Yasutomo K, Ishii E, Ohga S
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Journal Title
Am J Hematol
Volume: 87(6):637-9
DOI
Peer Reviewed
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[Journal Article] Hematopoietic stem cell transplantation with reduced intensity conditioning from a family haploidentical donor in an infant with familial hemophagocytic lymphohistocytosis2011
Author(s)
Ohta H, Miyashita E, Hirata I, Matsumura R, Yoshida H, Hashii Y, Higashiura T, Yasumi T, Murata Y, Heike T, Yang X, Kanegane H, Ohara O, Ozono K
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Journal Title
Int J Hematol
Volume: 94(3):285-90
DOI
Peer Reviewed
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[Journal Article] Rapid diagnosis of FHL3 by flow cytometric detection of intraplatelet Munc13-4 protein2011
Author(s)
Murata Y, Yasumi T, Shirakawa R, Izawa K, Sakai H, Abe J, Tanaka N, Kawai T, Oshima K, Saito M, Nishikomori R, Ohara O, Ishii E, Nakahata T, Horiuchi H, Heike T
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Journal Title
Blood
Volume: 118(5):1225-30
DOI
Peer Reviewed
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[Journal Article] Subtypes of familial hemophagocytic lymphohistiocytosis in Japan based on genetic and functional analyses of cytotoxic T lymphocytes2010
Author(s)
Nagai K, Yamamoto K, Fujiwara H, An J, Ochi T, Suemori K, Yasumi T, Tauchi H, Koh K, Sato M, Morimoto A, Heike T, Ishii E, Yasukawa M
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Journal Title
PLoS One
Volume: 5(11):e14173
DOI
Peer Reviewed
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