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トピックス 2016; 27 5 : 553-556 補体関連 TMA( 非定型 HUS) Complement-mediated TMA (atypical HUS) Masanori MATSUMOTO Key words: atypical HUS, TMA, complement, alternative pathway 3 511 6 11 716 6 16 719 9 19 1026 3 26 4 1 atypical hemolytic uremic syndrome, ahus 1 ahus C5 O157 shigatoxin producing E. coli, STEC HUS typical HUS HUS ahus ahus ahus= 2 HUS thrombotic thrombocytopenic purpura, TTP thrombotic microangiopathy, TMA 634-8522 840 Tel: 0744-22-3051 3289Fax: 0744-29-0771 E-mail: mmatsumo@naramed-u.ac.jp TMA TMA 1 2 TMA compliment-mediated TMA ahus ahus TMA ahus 2 3 C3 1TMA alternative pathway C3 C3bBb C3 C3b C3a a b b a C3b C3 C5 C5 27 5

554 1 TMA ADAMTS13 deficiency-mediated TMA ADAMTS13 TTP Complement-mediated TMA Coagulation-mediated TMA Metabolism-mediated TMA H I B C3 CD46 DGKE THBD MMACHC atypical HUS atypical HUS? ADAMTS13 deficiency-mediated TMA ADAMTS13 TTP Shiga toxin-mediated TMA STEC STEC-HUS Drug-mediated TMA immune reaction Drug-mediated TMA toxic dose-reaction TMA TMA Complement-mediated TMA H atypical HUS George 2 DGKE: diacylglycerol kinase ε THBD: MMACHC: methylmalonic aciduria and homocystinuria type C protein 1 3 TMA C3 H MCP C5b-9 TMA 9 MBL: mannose-binding lectin THBD: thrombomodulin TAFI: thrombin-activatable fibrinolysis inhibitor MCP: membrane cofactor protein C5b C5a C5b C6 C9 membrane attack complex, MAC H I MCP membrane cofactor protein, CD46 THBD C3b

555 2 ahus H n=95 H 20 30 7.4 H 5 10 10.5 C3 2 10 30.5 CD46 MCP 10 15 9.5 3 4 5.3 B 1 4 1.1 I 4 10 0 30 48.4 ahus 26 27 8 C3b Bb C3 C3 B C3bBb H I THBD thrombin-activatable fibrinolysis inhibitor TAFI C3a C5a 3 TMA TMA primarysecondary 2014 George TMA 9 2 1 4 5 ahus 1 TMA 2 H I MCP C3 B THBD THBD George DGKE diacylglycerol kinase ε coagulation-mediated TMA THBD in vitro C3b 3 ahus 2015 THBD TMA ahus DGKE 2015 ahus DGKE C3 4 TMA H 5 H 1 H Complement Factor H related, CFHR 1-5 CFHR3-CFHR1 H 6 CFHR H ahus DEAP-HUS deficiency of CFHR plasma proteins and autoantibody positive for of hemolytic uremic syndrome CFHR H 7 4 TMA ahus TMA 95 2 8 H 27 5

556 10 C3 30 48 TMA ahus ahus 9 C5 TMA 2016 DPC ahus E-mail ahusoffice@umin.ac.jp COI 1 Noris M, Remuzzi G: Atypical hemolytic-uremic syndrome. N Engl J Med 361: 1676 1687, 2009. 2 George JN, Nester CM: Syndromes of thrombotic microangiopathy. N Engl J Med 371: 1847 1848, 2014. 3 Delvaeye M, Noris M, De Vriese A, Esmon CT, Esmon NL, Ferrell G, Del-Favero J, Plaisance S, Claes B, Lambrechts D, Zoja C, Remuzzi G, Conway EM: Thrombomodulin mutations in atypical hemolytic-uremic syndrome. N Engl J Med 361: 345 357, 2009. 4 Miyata T, Uchida Y, Ohta T, Urayama K, Yoshida Y, Fujimura Y: Atypical haemolytic uraemic syndrome in a Japanese patient with DGKE genetic mutations. Thromb Haemost 114: 862 863, 2015. 5 Dragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, Weiss L, Fridman WH, Frémeaux-Bacchi V: Anti- Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 16: 555 563, 2005. 6 Loirat C, Frémeaux-Bacchi V: Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 6: 60, 2011. 7 Hofer J, Janecke AR, Zimmerhackl LB, Riedl M, Rosales A, Giner T, Cortina G, Haindl CJ, Petzelberger B, Pawlik M, Jeller V, Vester U, Gadner B, van Husen M, Moritz ML, Würzner R, Jungraithmayr T; German-Austrian HUS Study Group: Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 8: 407 415, 2013. 8 ahus 26 27 9 ahus 16: 223 231, 2015.