Profiles of Coagulation and Fibrinolysis Activation-Associated Molecular Markers of Atypical Hemolytic Uremic Syndrome in the Acute Phase
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- Sakurai Satoko
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
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- Kato Hideki
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
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- Yoshida Yoko
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
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- Sugawara Yuka
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
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- Fujisawa Madoka
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
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- Yasumoto Atsushi
- Department of Clinical Laboratory Medicine, the University of Tokyo Graduate School of Medicine
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- Matsumoto Masanori
- Department of Blood Transfusion Medicine, Nara Medical University
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- Fujimura Yoshihiro
- Japanese Red Cross Kinki Block Blood Center
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- Yatomi Yutaka
- Department of Clinical Laboratory Medicine, the University of Tokyo Graduate School of Medicine
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- Nangaku Masaomi
- Division of Nephrology and Endocrinology, the University of Tokyo Graduate School of Medicine
抄録
<p>Aim: Atypical hemolytic uremic syndrome (aHUS), characterized by thrombotic microangiopathy (TMA), is a genetic, life-threatening disease which needs many differential diagnoses. This study aimed to reveal coagulation and fibrinolysis profiles in aHUS and secondary TMA patients. Furthermore, we investigated whether aHUS patients progress to, and meet, disseminated intravascular coagulation (DIC) criteria. </p><p>Methods: The acute phase samples were available in 15 aHUS and 20 secondary TMA patients. We measured PT-ratio, activated partial thromboplastin time (APTT), fibrinogen, fibrin degradation product (FDP), fibrin monomer complex (FMC), antithrombin (AT), plasmin-α2 plasmin inhibitor complex (PIC), and von Willebrand factor antigen (VWF:Ag). We examined and compared these tests among aHUS, secondary TMA patients, and healthy volunteer (HV), and evaluated whether patients with aHUS and secondary TMA met DIC criteria.</p><p> Results: PT-ratio, APTT, FDP, FMC and PIC in patients with aHUS and secondary TMA were higher than those in HV. Fibrinogen and AT showed no significant difference among three groups. VWF:Ag was higher in only aHUS patients. No tests showed significant difference between aHUS and secondary TMA patients. Three aHUS patients out of 15 met DIC criteria.</p><p> Conclusion: We revealed the profiles and distributions of coagulation and fibrinolysis tests of aHUS and secondary TMA patients. All tests were enhanced compared to HV; however, our results showed the no specificities in distinguishing aHUS from secondary TMA patients. We also clarified that some aHUS patients fulfilled DIC diagnostic criteria, indicating that DIC itself cannot be an exclusion criterion of aHUS.</p>
収録刊行物
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- Ｊｏｕｒｎａｌ ｏｆ Ａｔｈｅｒｏｓｃｌｅｒｏｓｉｓ ａｎｄ Ｔｈｒｏｍｂｏｓｉｓ
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Ｊｏｕｒｎａｌ ｏｆ Ａｔｈｅｒｏｓｃｌｅｒｏｓｉｓ ａｎｄ Ｔｈｒｏｍｂｏｓｉｓ 27 (4), 353-362, 2020-04-01
一般社団法人 日本動脈硬化学会