DocHdl1OnPRS1tmpTarget

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1 2015; 26 6 : 自己免疫性出血病 FXIII/13 診断ガイド Guidance on Diagnostic Criteria for Autoimmune Hemorrhaphilia FXIII/13(AHFXIII/13) FXIII/13

2 659 Contents FXIII/ / 26 6

3 660 1 XIII/13 FXIII FXIII FXIII-A FXIII-A FXIII-B FXIII-B A 2 B 2 FXIII FXIII FXIII/13 Autoimmune Hemorrhaphilia FXIII/13 1 AHFXIII/13 2, 3 AHFXIII/13 FXIII/13 AHFXIII/ / F XIII/13 4,

4 661 4 AHFXIII/13 FXIII FXIII // FXIII 5 FXIII FXIII PT APTT FXIII 1 8 AHFXIII/13 FXIII FXIII-A FXIII-B 26 6

5 662 1 AHFXIII/13 1 SLE RA MGUS 5INH 6 7, 8 2 AHFXIII/ Schulman et al., 2005; Baudo et al., Hb 8 g/dl 2 g/dl Hb JBAT / 2010 Bleeding Assessment Tools ver , 5 7, AHFXIII/13 3 AHFXIII/13 FXIII FXIII FXIII FXIII FXIII FXIII FXIII FXIII FXIII AHFXIII/13 FXIII 4

6 663 3 FXIII/13 AHFXIII/ definite probable possible AHFXIII/ / XIII FXIII FXIII 50 probable FXIII 37 C 2 definite FXIII ELISA 1 XIII/13 FXIII 2 FXIII FXIII/13 Autoimmune Hemorrhaphilia FXIII/13; AHFXIII/ PT aptt 2 FXIII FXIII FXIII FXIII AHFXIII/13 DIC FXIII FXIII / FXIII-A FXIII-B FXIII-A FXIII-B FXIII-A 2 B 2 FXIII / 26 6

7 664 AHFXIII/13 4 FXIII 1 DIC

8 665 3 FXIII FXIII FXIII FXIII FXIIIa FXIII FVIII/8 Bethesda FXIII FXIII AHFXIII/13 / FXIII ELISA FXIII FXIII 4 / FXIII AHFXIII/13 AHFXIII/13 FXIII FXIII γ α γ α α 2 -plasmin inhibitor α 2 -PI α 2 -PI α 2 -PI FXIII 50 α 2 -PI AHFXIII/13 FXIII FXIII AHFXIII/13 COI COI 26 6

9 Ichinose A: Japanese criterion 2012 for the diagnosis and treatment of acquired hemorrhaphilia XIII/13. 58th Annual Meeting of the Scientific and Standardization Committee (SSC) of the International Society on Thrombosis and Haemostasis (ISTH), June 27 30, 2012, ACC Liverpool (Liverpool, England); Ichinose A: An update on Japanese criterion 2012 for the diagnosis and treatment of autoimmune/ acquired hemorrhaphilia XIII/13; a proposal of algorithm of laboratory tests and differential diagnosis. XXIV Congress of the International Society on Thrombosis and Haemostasis, 59th SSC Program, Subcommittee session, June 29 July 4, 2013, Amsterdam RAI (Amsterdam, The Netherlands) 2 Lorand L. Acquired Inhibitors of Fibrin Stabilization: A Class of Hemorrhagic Disorders of Diverse Origins. In: Green D ed. Anticoagulants Physiologic, Pathologic, and Pharmacologic. CRC Press, 1994: Egbring R, Kröniger A, Seitz R: Erworbene inhibitoren gegen Faktor XIII. Hamostaseologie 16: , Ichinose A; Japanese Collaborative Research Group (JCRG) on AH13 Hemorrhagic Acquired Coagulopathies: Inhibitors of Factor XIII/13 in older patients. Semin Thromb Hemost 40: , Factor XIII 25: , Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P, Neunert C, Lillicrap D; ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group: ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 8: , Schulman S, Kearon C; Subcommittee on Control of Anticoagulation of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis: Definition of major bleeding in clinical investigations of antihemostatic medicinal products in non-surgical patients. J Thromb Haemost 3: , Baudo F, Collins P, Huth-Kühne A, Lévesque H, Marco P, Nemes L, Pellegrini F, Tengborn L, Knoebl P; EACH2 registry contributors: Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood 120: 39 46, Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L; Factor XIII And Fibrinogen SSC Subcommittee Of The ISTH: Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost 9: , Muszbek L, Ariëns RA, Ichinose A; ISTH SSC SUBCOMMITTEE ON FACTOR XIII: Factor XIII: recommended terms and abbreviations. J Thromb Haemost 5: , 2007.

10 667 以下は補足的な参考情報である附1 第 26 巻第 6 号日本語版標準化出血質問票出血評価票 JBAT

11 668 2 FXIII / FXIII FXIII FXIII-A 9 FXIII-B FXIII FXIII-A 10 A B FXIII FXIII:Agn FXIII-A FXIII-B FXIII-A FXIII-A 2 FXIII-B FXIII-B 2 FXIII FXIII-A 2 B 2 FXIII pfxiii FXIII cfxiii FXIII FXIII:Act FXIII FXIIIa

DocHdl1OnPRS1tmpTarget

DocHdl1OnPRS1tmpTarget 2017; 28 3 : 393-420 自己免疫性出血病 FXIII/13 診療ガイド Guidance on Diagnosis and Management of Autoimmune Hemorrhaphilia FXIII/13(AHFXIII/13) FXIII/13 0.0 28 2 28 1.0 28 4 18 1.1 28 9 5 2.0 28 11 30 3.0 28 12 20