Development of novel methods for diagnosis and management of thrombotic microangiopathy following stem cell transplantation
| Project/Area Number |
25461456
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Hematology
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| Research Institution | Nara Medical University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
HAYAKAWA Masaki 奈良県立医科大学, 輸血部, 助教 (30516729)
藤村 吉博 奈良県立医科大学, 医学部, その他 (80118033)
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| Project Period (FY) |
2013-04-01 – 2016-03-31
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| Project Status |
Completed (Fiscal Year 2015)
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| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 造血幹細胞移植 / 血栓性微小血管症 / TMA |
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| Outline of Final Research Achievements |
For improving the prognosis of thrombotic microangiopathy following hematopoietic stem cell transplantation (SCT-TMA), we analyzed the pathophysiology focusing on von Willebrand factor (VWF) and its cleaving protease ADAMTS13. In the TMA registry in Japan, the incidence of decreased ADAMTS13 activity under 10% of normal control in patients with SCT-TMA (4/89, 4.5%) was low compared with those of acquired primary thombotic thrombocytopenic purpura (347/420, 82.6%). There was no patient with ADAMTS13 activity under 0.5%. We found the appearance of unusually large VWF multimers, which are the most active form of VWF, just before development of SCT-TMA. Moreover, high moleculer weight VWF multimers were lacked during the events of SCT-TMA. We proposed the novel diagnostic criteria for early diagnosis of SCT-TMA including serum haptoglobulin, corrected count increment of platelet which can calculate using platelet counts before and after platelet transfusion.
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Report
(4 results)
Research Products
(23 results)
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[Journal Article] STEC:O111-HUS complicated by acute encephalopathy in a young girl was successfully treated with a set of hemodiafiltration, steroid pulse, and soluble thrombomodulin, under plasma exchange.2015
Author(s)
Yada N, Fujioka M, Bennett CL, Inoki K, Miki T, Watanabe A, Yoshida T, Hayakawa M, Matsumoto M, Fujimura Y.
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Journal Title
Clin Case Rep.
Volume: 3
Issue: 4
Pages: 208-212
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Identification of N-linked glycosylation and putative O-fucosylation, C-mannosylation sites in plasma derived ADAMTS13.2014
Author(s)
Sorvillo N, Kaijen PH, Matsumoto M, Fujimura Y, van der Zwaan C, Verbij FC, Pos W, Fijnheer R, Voorberg J, Meijer AB.
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Journal Title
J Thromb Haemost
Volume: 12
Pages: 670-679
Related Report
Peer Reviewed
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[Journal Article] Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: Concentration of C3 p.I1157T mutation.2014
Author(s)
Matsumoto T, Fan X, Ishikawa E, Ito M, Amano K, Toyoda H, Komada Y, Ohishi K, Katayama N, Yoshida Y, Matsumoto M, Fujimura Y, Ikejiri M, Wada H, Miyata T.
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Journal Title
Int J Hematol.
Volume: 100
Issue: 5
Pages: 437-442
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndorome) on hemodialysis for 19 years: a case report2013
Author(s)
Mise K, Ubara Y, Matsumoto M, Sumida K, Hiramatsu R, Hasegawa E, Yamanouchi M, Hayami N, Suwabe T, Hoshino J, Sawa N, Ohashi K, Kokame K, Miyata T, Fujimura Y, Takaichi K.
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Journal Title
BMC Nephrol.
Volume: 14
Issue: 1
Pages: 156-156
DOI
Related Report
Peer Reviewed
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[Book] ADAMTS132015
Author(s)
Fujimura Y, Kokame K, Yagi H, Isonishi A, Matsumoto M, Miyata T
Total Pages
184
Publisher
Springer
Related Report
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