2024-03-28T13:36:05Z
https://niigata-u.repo.nii.ac.jp/oai
oai:niigata-u.repo.nii.ac.jp:00021282
2022-12-15T04:16:15Z
453:456
471:537:538:1182
4) 慢性骨髄増殖性疾患におけるvon Willebrand因子異常(新潟血栓止血研究会10周年記念特別例会記録)
4) 慢性骨髄増殖性疾患におけるvon Willebrand因子異常(新潟血栓止血研究会10周年記念特別例会記録)
Abnormalities of Plasma von Willebrand Factor in Patients with Chronic Myeloproliferative Disorders
帯刀, 亘
高橋, 芳右
柴田, 昭
von Willebrand factor
chronic myeloproliferative disorders
in vivo proteolysis
von Willebrand因子
慢性骨髄増殖性疾患
生体内蛋白分解
In chronic myeloproliferative disorders (CMPD) hemorrhagic complications are relatively common, in association with thrombocytosis. We studied the multimeric composition of plasma von Willebrand factor (vWf) in CMPD and investigated the possibilities of in vivo or ex vivo proteolysis. The relative amount of large multimers in patients with CMPD was significantly decreased in relation to platelet count as compared with normal subjects. It correlated negatively with platelet count, and positively with the ristocetin cofactor/vWf antigen ratio. All patients with primary myelofibrosis who had normal or subnormal platelet counts showed an almost normal multimeric pattern. The vWf abnormalities normalized with decreasing platelet counts after starting chemotherapy or when blast crisis occurred with decreased platelet counts. These abnormalities would be caused by in vivo proteolysis, because they were not corrected even when blood was obtained in the presence of protease inhibitors. The relative amount of large multimers of vWf did not correlate with plasma concentration of plasmin or elastase. Thus neither plasmin nor elastase appear to be the enzymes responsible for in vivo proteolysis.
新潟医学会
1991-09
jpn
departmental bulletin paper
http://hdl.handle.net/10191/39396
https://niigata-u.repo.nii.ac.jp/records/21282
AN00182415
00290440
新潟医学会雑誌
新潟医学会雑誌
105
9
628
631
https://niigata-u.repo.nii.ac.jp/record/21282/files/105(9)_628-631.pdf
application/pdf
847.9 kB
2019-08-16