災難性抗磷脂症候群 - tsim.org.tw

1 2009 20 335-343 114 2 325 1,2 21 2 (antiphospholipid syndrome) (lupus anticoagulant LA) (anti-cardiolipid IgG/IgM antibodies) 2 I (anti- 2-glycoprotein I antibodies anti- 2 GPI ) (inflammatory bowel dise-ase) 1 2-4 (bone marrow necrosis) (thrombotic thrombocytopenic purpura) (hemolytic uremic syndrome) HELLP ( ) (catastrophic antiphospholipid syndrome) 5,6 1992 Asherson R.

2 A. Asherson's syndrome7 (catastrophic antiphospholipid syndrome) 1% Warfarin 50% (Catastrophic antiphospholipid syndrome) (Thrombosis) (Multiple organ failure) (Thrombotic storm)336 8 9 1% 51% 10 ( Protein C, Protein S Anti-thrombin III ) 8 (inhibition of anticoagulant reaction) (monocyte) (endothelial cell) 11 (tissue factor) ICAM-I (adhesion mole-cules) (procoagulant activity) (fibrinolysis)

3 Thromboxan A2 protein C antithrombin 2 GPI annexin A5 2 GPI phosphatidylserine Signal trans-ducer and activator of transtription 5 (Stat5) 12,13 ( 50,000 /mm3) (1) (2) (membrane glycoproteins: GPIIb/IIIa, GPIb/IX, GPIV) (3) 5 8,14 14 2 1998 Kitchens (thrombotic storm)

4 15 plasminogen activator inhibitors Protein C Protein S Antithrombin III 40% 22% 14% 3% 337 16 8 40% 1 HIV B C 17 40% 4 ( 1%) 16 10% LA 18.

5 19 18 8 15,16 HELLP ( ) 19 captopril, danazol, thiazide diuretics 1,16 16 338 13% (disseminated intravascular coagulation) (fibrinogen degradation products) D-dimers (fibrinogen) (prothrombin time) 20 (thrombotic microan-giopathic hemolytic anemia TMHA)

6 26% 23% 15% 13% 13% HELLP 4%21 TMHA 8 (confusion) (disorientation) (drowsiness) (stupor) (mononeuritis multiplex ) 66% (livedo reticularis) 8 53% 8 (acute respiratory distress syndrome, ARDS) ARDS 20% ARDS 32 ( ) 26 339 (hyaline membrane) (systemic infl-ammatory response syndrome SIRS) SIRS ( tumor necrosis factor- , interlukin-1, interlukin-6, macrophage migration inhibitory factor) (surfactant)

7 ARDS 22 8 TMHA TMHA (HELLP ) TMHA 61% 22 TMHA TMHA (heparin) (unfraction-ated hearpin) (low molecular weight heparin) LA anticardio-lipid antibodies anti- 2 GPI Thrombotic microangiopathic hemolytic anemia(TMHA)

8 / / / 340 INR(International Normalized Ratio) 2 3 warfarin Warfarin INR INR 2 3 23 aspirin clopidogrel aspirin clopidogrel aspirin HydroxychloroquineHydroxychloroquine hydroxychloroquine 24,25 methylprednisolone 1 g prednisone 1-2 mg/Kg 8 (plasma exchange)

9 (cryoglobulinemia) 21,26 (intravenous immuno-globulins IVIG) g/kg/d 8,16 Rituximab CD20 B 27,28 Cyclophosphamide 16 DefibrotideDefibrotide tumor necrosis factor- 29 Fibrinolytic agent ancrol, streptokinase, uro-kinase.

10 Tissue plasminogen activators Prostacyclin 5 ng/Kg/min Rituximab ( cyclophosphamide) ( prostacyclin 341ancrod defibrotide) 16 16 50% (ARDS) (20%) (14%) (14%) (12%)16,30,31, 31 60% 20% 20% 30 (ANA) 31 50% 14,32 30 ( )