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特集 : 自己抗体による出血血栓 : 基礎と臨床の現状 2018; 29 3 : 273-280 後天性フォンヴィレブランド症候群 Acquired von Willebrand Syndrome (AvWS) Nobuaki SUZUKI 要約 :von Willebrand Factor VWF Acquired von Willebrand Syndrome AvWS 1968 SLE

1 特集 : 自己抗体による出血血栓 : 基礎と臨床の現状 2018; 29 3 : 後天性フォンヴィレブランド症候群 Acquired von Willebrand Syndrome (AvWS) Nobuaki SUZUKI 要約 :von Willebrand Factor VWF Acquired von Willebrand Syndrome AvWS 1968 SLE 50 VWF RCo ELISA VWF AvWS VWF VIII Key words: Acquired von Willebrand Syndrome, von Willebrand Factor, inhibitor 1. はじめに Acquired von Willebrand Syndrome AvWS von Willebrand Factor VWF Tel: Fax: suzukin@med.nagoya-u.ac.jp 1968 Simone J SLE 50 VWF VWF: RCo ELISA

2 274 1 B MGUS MPN MPN AS VSD MGUS: monoclonal gammopathy of unknown significance, MPN: myeloproliferative neoplasms, AS, VSD AvWS 2. 疫学的背景と基礎疾患 1998 International Society Thrombosis and Hemostasis ISTH 1 AvWS 62 1 AvWS 2 myeloproliferative neoplasms: MPN aortic stenosis: AS gastrointestinal angiodysplasia AS high shear stress VWF a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 ADAMTS13 VWF VWD type2a 2 AvWS 病態 AvWS VWF: RCo VIII FVIIIVWF FVIII FVIII FVIII FVIII: C VWF 3, 4 MPN AvWS VWF 2 VWD 5 7

3 275 2 AvWS ISTH n=186 n= MGUS CLL ALL 1 0 myeloproliferative neoplasms: MPN CML AvWS AvWS

4 276 3 Lazarchick J AvWS VWF IgG 8 MPN AvWS AvWS 4. 臨床所見 3 AvWS MPN 2 MPN: myeloproliferative neoplasms 1 VWF/FVIII 25 20/80 4 1/ /8 33 1/2 5 2/39 7 1/14 VWF: RCo FVIII VWF FVIII 9 VWF 5. 診断法 VWF VWD VWF VIII 2 PT APTT AvWS PT APTT or 2 APTT FVIII FIX VWF: RCo VWF PT APTT VWF: RCo VWF FXIII XIII APTT FVIII APTT APTT AvWS A A FVIII VWF: RCo VWF APTT AvWS A VWF VWF AvWS VWF VWF: RCo VWF: RCo/ VWF <0.6 VWF

5 VWD VWD AvWS VWD O VWF ELISA VWF: RCo 10 VWF-propeptide VWF VWF 11 VWD VWF: RCo VWF FVIII VWF 6. 治療法 2011 Blood How I treat 4 7. 単クローン性ガンマグロブリン血症 (monoclonal gammopathy of undetermined significance: MGUS)

6 278 4 MGUS B SLE ITP APS IgG MGUS IgM MGUS g/kg VWF 3 VWF VIII FVIII IU/kg μg/kg VWF 13 MGUS MGUS VWF: RCo 14 IgG- MGUS intravenous immunoglobulin IVIG IgM- MGUS 11 8.B 細胞性リンパ腫および多発性骨髄腫 AvWS VWF VWF: RCo 6, 7 AvWS , 15 VWF 9. 骨髄増殖性腫瘍 (MPN) MPN 11 AvWS 15 MPN AvWS essential thrombocythemia: ET

7 279 VWF: RCo VWF Mohri AvWS MPN /μl AvWS VWF: RCo 10. 自己免疫疾患 IVIG 11 AvWS 2 Lee A SLE AvWS 17 idiopathic thrombocytopenic purpura: ITP APS ITP ITP AvWS 1 18 AvWS PSL 1.0 mg/kg/day VWF: RCo 0.16 mg/kg/day 19 AvWS 11. 急性出血に対する治療 VWF VIII AvWS , その他の出血時治療 11 VII まとめ AvWS AvWS 29 3

8 280 COI 1 Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE; Subcommittee on von Willebrand Factor: Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 84: , : , Mannucci PM, Lombardi R, Bader R, Horellou MH, Finazzi G, Besana C, Conard J, Samama M: Studies of the pathophysiology of acquired von Willebrand s disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies. Blood 64: , van Genderen PJ, Vink T, Michiels JJ, van t Veer MB, Sixma JJ, van Vliet HH: Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 84: , Budde U, Schaefer G, Mueller N, Egli H, Dent J, Ruggeri Z, Zimmerman T: Acquired von Willebrand s disease in the myeloproliferative syndrome. Blood 64: , Richard C, Cuadrado MA, Prieto M, Batlle J, López Fernández MF, Rodriguez Salazar ML, Bello C, Recio M, Santoro T, Gomez Casares MT, et al. Acquired von Willebrand disease in multiple myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol 35: , Budde U, Scharf RE, Franke P, Hartmann-Budde K, Dent J, Ruggeri ZM: Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 82: , Lazarchick J, Pappas AA, Kizer J, Hall SA: Acquired von Willebrand syndrome due to an inhibitor specific for von Willebrand factor antigens. Am J Hematol 21: , : 82 90, Tiede A, Priesack J, Werwitzke S, Bohlmann K, Oortwijn B, Lenting P, Eisert R, Ganser A, Budde U: Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost 6: , Tiede A, Rand JH, Budde U, Ganser A, Federici AB: How I treat the acquired von Willebrand syndrome. Blood 117: , Grimaldi D, Bartolucci P, Gouault-Heilmann M, Martin- Toutain I, Khellaf M, Godeau B: Rituximab failure in a patient with monoclonal gammopathy of undetermined significance (MGUS)-associated acquired von Willebrand syndrome. Thromb Haemost 99: , Lavin M, Brophy TM, Rawley O, O Sullivan JM, Hayden PJ, Browne PV, Ryan K, O Connell N, O Donnell JS: Lenalidomide as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy. J Thromb Haemost 14: , Wolfe Z, Lash B: Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. Case Rep Hematol 2017: , Mohri H, Motomura S, Kanamori H, Matsuzaki M, Watanabe S, Maruta A, Kodama F, Okubo T: Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 91: , van Genderen PJ, Michiels JJ, van der Poel-van de Luytgaarde SC, van Vliet HH: Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: relationship with platelet count. Ann Hematol 69: 81 84, Lee A, Sinclair G, Valentine K, James P, Poon MC: Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. Blood 124: e1 3, ITP VWF von Willebrand 56: , von Willebrand 58: , Tiede A: Diagnosis and treatment of acquired von Willebrand syndrome. Thrombosis research 130 Suppl 2: S2 6, Franchini M, Veneri D, Lippi G: The use of recombinant activated factor VII in congenital and acquired von Willebrand disease. Blood Coagul Fibrinolysis 17: , 2006.

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DocHdl1OnPRS1tmpTarget 2015; 26 6 : 658-668 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/13 659 Contents 1 660 2 660 3 660 4 661 5 661 6 661 7 661 8 661 9 662