WORLD FEDERATION OF HEMOPHILIA
World Federation of Hemophilia 2008 WFH www.wfh.org WFH World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA (514) 875-7944 (514) 875-8916 wfh@wfh.org www.wfh.org WFH Man-Chiu POON
1 3 3 3 4 4 4 4 5 5 6 6 7 7 7 7 8 8 9 9 10 10 1. 11 2. B 11 3. 11 4. 12 / 12 12 13 13 14 1.. 14 2. 14 3. 15 15 16 16
17 17 17 17 17 1. 17 2. 18 3. PPP 18 4. 18 19 19 19 20 20 20 21 21 EQAS 22 22 23 23 23 24 25 25 25 26 26 27 28 29 29 30 1. 30 2. 31 31 31
32 32 DDAVP 32 34 34 37 37 A FVIII 37 FVIII 37 /FFP 38 DDAVP 38 B FIX 38 FIX 38 FFP 39 40 40 40 40 40 / 41 41 41 41 42 42 42 42 43 43 43 43 44 44 44 45 47
1 30 WFH 2003 Paul Giangrande Man Chiu Poon Mary Chua A McCraw Jerome Wiedel Alok Srivastava WFHElizabeth Myles WFH WFH Alok Srivastava, MD WFH
3 X- VIII FVIII AIX FIXB 400 000 A B80% 85% FVIII FIX 1/3 APTT 1 1
1-1 % (IU/ml) 1% <0.01 1% 5% 0.01 0.05 5% 40% 0.05 0.40 / // 70 80% / 10 20% 5 10% <5% 45% 3% 30% 2% 15% 2% 3% 4
5 FVIII/FIX HIV HBV HCV HAV) B19 X- <30%
FFP / 6
7 161 2h /
8 DDAVP (ASA) NSAIDs, COX-2 A DDAVPFVIII RICE( ) DDA 5 10d
9 COX-2 Port-A-Cath 1% 8 25 40IU/kg A 3B 2
- 1 1-3 45 10% 15% A1% 3% B 10 20d <5 BU 5BU 3 12 10 20 10
Bethesda 1. 5d 5BU a FEIBA Auto 2. B B FIX ) 3. A ITI ITIFVIII FEIBA ITI 3 200 IU/kg B ITI BFIX ITI 11
4. FVIII / CVS 10 11 12 15 RhD D VIII 50% 50% VIII 12
HIV HIV 13
14 1. 2.
3. 2 1213 AFVIII>5% DDAVP 5%1 2min4 7d 15
NSAIDs 5 10d, 6 12 HIV HCV HBV 16
WFH BT PT(AP 1. 17
2. 1h 30min 1min 19 21 SWG 22 23 SWG 2ml 3.2% 3.8% 9: 1 PPP4 C2h -30 C PPP 2 000g 3500r/min15mi 4 C PPP20 25 C 4 C 2hPPP -30 C 3. 37 C 18
BT PT APTT 1-2 FXIII PAI-1 1-2 PT APTT BT A B VWD NPP FVIII FIX FVIII/FIX WFH 19
FVIII 80 U FVI FVIII C/VWF:Ag 1.0 0.7 80% WFH FVIII C/VWF:Ag WFH WFH WFH 37 ± 0.5 C 0.1ml 0.2ml 20
21 7.5cm 1.2cm QA QA (IQC) IQC
QA EQAS QA EQAS WFH/WHO EQAS WHO 20 10 20ml 2 000g15min4 C 500ul-70 C 37 C WHO NIBSCWHO 22
<1% 1%~5% 2 10 20 1-345 12 24h 23
12h, NSAIDs COX-2NSAIDs 48 72h 30 50%48 72h 16 1h 24
Volkmann 1-3 45 10 20 X MRI 6 8 NSAIDs COX-2 25
26 32 90 10 20 /
NSAIDsCOX-2 serial casting bracing orthotics X CTMRI 27
50%3 5d 10 14d 28
29 FVIII FIX Dr.Car 1997 WFH PCR WFH WFH HB HCV HIV GMPs NAT ;
GMPs HIV HBV HCV HAV B19 Creutzfeldt-JakobvCJD vcjd vcjd / 1. FVIII 10 IU 10 100 IU 100 1 000 IU 1 000 IU FII FVII FX FIX 30
FVIII 2. HIV HAV HCV HCV HIV HAV B19 WFH FFP4 C 10 24h FVIII5 IU/ml VWFFXIII FIX FXI FVII FIX FX FXI WFH 31
HIV HBsAg PCRHIV HBV HCV FVIII FFP FFPFIX B FVFFP FFP FXI FFP/ / FFPWFH FFP DDAVP DDAVP DDAVPADH FVIII VWF 32
0.3mg/kg1FVIII3 6 90min DDAVP 1 2d A DDAVPFIX B FVIII FVIII10% 30 40% DDAVP DDAVP DDAVP DDAVP50 100ml, 2 2DDAVP DDAVP DDAVP DDAVP 0.3mg/kg50kg 50kg 150mg DDAVP 33
FXI 3 4g,, 5ml 500mg 25mg/kg75mg/kg, APCCsFEIBAAutoplex APCC 4 6h FVIIa 34
5g1g8 250mg/ml 50 100mg/kg5g 35
2h 23 25 3 5min NSAIDs 2 3 FVIII FVIII FVIII 250 2 000U 37
1U/kg FVIIIFVIII 2% kg 50kg 40 % 0.5=1 000U FVIII 45 FVIII 3ml 100U VIII 24 48h, 24 48h / FFP 30 40mlFVIII60 100U FFPFFP 1ml FFP 1U DDAVP FVIII5% DDAVP B FIX FIX FIX FIX FIX PCCs 38
FIX FIX DIC FIX 300 1 200U 1U/kg FIX1% FIX BeneFIX Wyeth1U/kg 0.8%150.7%FIX 50kg 40 %=2 000UFIX 2 000 0.82 000 1.25 =2 500U 2 000 1.43 =2 860U 45 FIX 3ml 100U PCC APCC 24 48h 24 48h FFP B FIX FIX 25% /FFP 39
X 1-3 45 A 12hB 24h 3d,, 1-3 45 2 3d ( ) 1-3 45 4 96h1-3 45 CT 40
100 mg / 1-3 45 1-3 45 2 3 CTMRI 1-3 45 1-3 45 FVIII FIX 1-3 45 41
1-3 45 3L/m 2 48h 1-3 45 FIX FVIII 42
/ 1-3 45 DDAV DDAVP A 50% 40% 1-3 45 5 5 10d 24h24g 6 8h 110d 43
/ 1-3 45 5 7d 10 14d1-3 45 44
1-3 1-3 A B 40 60% 1 2 40% 60% 1 2 40% 60% 2 3 40% 60% 2 3 80% 100% 1 2 60% 80% 1 2 30% 60% 3 5 30% 60% 3 5 CNS/ 80% 100% 1 7 50% 8 21 80% 100% 1 7 50% 8 14 60% 80% 30% 60% 80% 30% 1 7 8 21 1 7 8 14 80% 100% 1 6 50% 7 14 60% 80% 30% 1 6 7 14 50% 3 5 40% 3 5 50% 5 7 40% 5 7 80% 100% 60% 80% 40% 60% 30% 50% 1 3 4 6 7 14 60% 80% 40% 60% 1 3 30% 50% 4 6 20% 40% 7 14 45
A B 10% 20% 1 2 10% 20%1 2 10% 20% 2 3 10% 20%2 3 20% 40% 1 2 15% 30% 1 2 10% 20% 3 5 10% 20% 3 5 CNS/ 50% 80% 1 3 30% 50% 4 7 50% 80% 1 3 30% 50% 4 7 20% 40% 8 1421 20% 40% 8 1421 30% 50% 10% 20% 1 3 4 7 30% 50% 1 3 10% 20% 4 7 30% 50% 1 3 10% 20% 4 7 30% 50% 1 3 10% 20% 4 7 20% 40% 3 5 15% 30%3 5 20% 40% 5 7 60% 80% 30% 40% 1 3 20% 30% 4 6 10% 20% 7 14 15% 30%5 7 50% 70% 30% 40% 1 3 20% 30% 4 6 10% 20% 7 14 46
Association of Hemophilia Clinic Directors of Canada. Clinical practice guidelines:hemophilia and von Willebrand s disease: 2.Management. Edition 2, Update. 1999. Association of Hemophilia Clinic Directors of Canada. Hemophilia and von Willebrand s disease: 1. Diagnosis, comprehensive care and assessment. Edition 2. Update. 1999. Farrugia, A. Guide for the assessment of clotting factor concentrates for the treatment of hemophilia. World Federation of Hemophilia. 2003. Hemophilia of Georgia. Protocols for the treatment of hemophilia and von Willebrand disease. 2004. Kitchen, S. and Angus McCraw. Diagnosis of hemophilia and other bleeding disorders: A laboratory manual. World Federation of Hemophilia. 2000. Karabus, C, ed. Treatment guidelines for hemophilia in South Africa. South African Hemophilia Foundation. Kasper, C.K.,and Meirione Costa e Silva. Registry of clotting factor concentrates. Fifth edition. World Federation of Hemophilia. 2004. National Hemophilia Foundation. Standards and criteria for the care of persons with congenital bleeding disorders. 2002. Santagostino, E., P.M. Mannucci, and A. Bianchi bonomi. Guidelines for replacement therpy for hemophilia and inherited coagulation disorders in italy. Hemophilia. 2000.6:1-10. Srivastava, A., ed. Guidelines for management of hemophilia in India. Hemophilia Federation (India). 47
World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA (514) 875-7944 (514) 875-8916 wfh@wfh.org www.wfh.org