2015 Fiscal Year Final Research Report
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 |
Section | 一般 |
Research Field |
Hematology
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Research Institution | Nara Medical University |
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)
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Project Period (FY) |
2013-04-01 – 2016-03-31
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Keywords | 造血幹細胞移植 / 血栓性微小血管症 |
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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Free Research Field |
血液内科
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