| 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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