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5

Wszystko o hemofilii

Poradnik dla rodziny

5–2



Leczenie czynnikiem !"#$%&'(&)5

n 8/+,/+2#3,+1#("#%&#+("-%%& &#.+ !"#$%&'(&)9

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5–3




l C%KQZM%2<%#2%[&#!7+%H--/%-#)2$E*5%:"%('$44!)%WJJJ%1";7%A*2#'-%;7>:-4$%3%)2!-92"($9!7*(!"5%('$/!%-;*#'!"5%)782$%9-3;7*1"5%)!"#$ 3%-#9-3!"#4!%;9-;8A%2-',2*:*%;!>%-;-('"<%=-%-#)2$(!"%;9-3-#-3*E-%2"3-/&(1>%3%/"('"4!&%+",-./!!%\<%]-:4*%A$E-%92'"4!"DG%-9!")>%4*#%9*(1"47*,!%'"%;'9!7*/!%#-%92'$(+-#4! !%#-,83<%

l C%98^4$(+%/*7*(+%ZN<%!%3('";4$(+%_N<%3$92-#&)-3*4-%)-4("472*7$%('$44!)83%)2'"94!>(!*%9-92'"'%-##'!"/"4!"%-#%;!"A!"%28:4$(+%A!*E")%)2'"94!>(!*%'4*1#&10($(+%;!>%3%-;-('&<%TA1>7-DG%)-4("472*783%('$44!)*%A$E*%4!"3!"/)*5%/"('%'*3!"2*E$%-4"%#&:0%!/-DG%*)7$34"6-%A!*E)*%@('$44!)*%)2'"94!"(!*B5%#/*7"6-%-)*'*E$%;!>%'4*('4!"%;)&7"('4!"1;'"%3%'*72'$,$3*4!&%)23*3!"S%4!:%)2!-92"($9!7*7<%`$E$%-4"%7":%3$6-#4!"1;'"5%9-4!"3*:%1*)-%-;&;'-4"%@/!-./!'-3*4"B%;&A;7*4(1"%3%O-2,!"%92-;')&%,-6E$%A$G%72'$,*4"%3%/-#83("5%*%4*;7>94!"%2-'9&;'('*4"%3%;7"2$/4"1%3-#'!"5%)!"#$%3$;709!E*%)-4!"('4-DG%!(+%&:$(!*<%T'4*('*%7-5%:"%-;-A*%(+-2*%4*%+",-./!>%,-6E*%1"%4-;!G%92'$%;-A!"<%a'!>)!%7",&%(+-2'$%,-6/!%'*('0G%9-#28:-3*G<%

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3%/*7*(+%_N<%!%LN<%92"9*2*783%)23!-9-(+-#4$(+%;9-3-#-3*E-%'*2*:"4!*%3!2&;*,!%'*9*/"4!*%3072-A$%7$9&%`%!%F%-2*'%3!2&;",%bJW<%TA"(4!"%)-4("472*7$%('$44!)*%3$92-#&)-3*4"%'%-;-('*%&3*:*4"%;0%'*%A"'9!"('4"%!%3-/4"%-#%3!2&;83%'*9*/"4!*%3072-A$%`%!%F%-2*'%bJW<% + !"#$%"&'()#*+,-./#+01234(-/+:#%2!(-% #"$%&)'2%.'34",$45"64'78'

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5–4




Krew osoby chorej

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Tabela 1

S&3,/!&)+1#("#%&)+("-%%& &#.+ !"#$%&'(&)+4+:)%)0"&#

1947

1965

19681969

1985

19871987

19881991

D.!4"<.&8$,B") !4-:5!8+)"5).B,?5

D.!4"<.&8$,B") !4-:5!8+)"5).B,?5

D.!4"<.&8$,B") !4-:5!8+)"5).B,?5

A !4-:5!8+!"5).B,?5"2&.=8:%"<&5)$-./$%

A !4-:5!8+!"5).B,?5"/!"<&%&8="2&.=8$+"<!.:"5&0 $+1"<!,-$.:/!8+)1"%)$,/! ) :".&/<B,/8/!( )4!")"0$-$.2$ -B

N8/:,/8/! :"<./$8)58)!;!%)"%& &4(& !( :%)

O5)$3!"<$; !"4.$5O5)$3&"%.&3& $"&,&8/$

P.)&<.$8:<)-!-"J8/: )4"@AAAK

!"#$"%"#&"'()*+"(#)(,-.,/*

#$/((0+.*+-$)%(01#0.*

230"430$"5.()6

!"#$"%&'%(#)*""+,'(-...

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!"#$"%"#&"'()*+"(#)(,-.,/*

#$/((0+.*+-$)%(01#0.**

230"43"$"5.()6*$*0(.+,/5.#78*

50-9!:5

!"#$"%&'%*(#)*""+,01(-...(+(./

;"(#)(,-.,/*#$/((0+.*

"!"#$"%"#&"'()<"*

230"430$"5.(/6*=*$)*$501+!$"(8*

0(.+,/5.#78*50-9!:5

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2*3!,!(!#)*3)#)!"*(

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2*3!,!(!#)*3)#)!"*(#)*""+,(./

>-"'9+,**

5–5



Leczenie czynnikiem +-$)%(01#0.5

?"+** * * >-"'9+,** @(.+,/5.#7.*50-9!:5

Tabela 1

A0!,"-0.*3)#$)(0.*#$/((0+0)B*+-$)%(01#0.*5*;.(.'$0)*2#C'C6

1993

1997

2000

2006

2008

456),+$(+()1+$&)7#$(8+'9,' 1(:!6!1;+(,!<0&,!1$=>(?&!65,%(,!@#!1*()'1+$&'(;56),A(';85<+"7(B3%'8+;+)'%!&C>

D!6!1;'(,!<0&,!1'>(E#)*3)#)'"+$(+(3%'8+;+)!1'"+$(8$)(;56),+#:(;58()1+$&)7#*#:(8+'9$,>(

F+'9,'()(;56),+$G!(!3!#)' 1(:!6!1;+(,!<0&,!1$= +(8+'9,!(<*3)*(H!%&)$8"$ 6!($%'H5(!#)*3)#)'"+'(#)*""+,'(-...(H!6#)'3(H&!65,#=+I(';$(3%'8+;+)'#='(H&!65,%5(,!@#!1$G! )'(H!<!#A(3'#:'&!)*()'<+'3%(';85<+"*>

F&',(;56),+#:(8+'9$, 1(H&!#$3+$(:!6!1;+(,!<0&,!1$=>(J%'H(!#)*3)#)'"+'(#)*""+,'(-...(H!6#)'3(H&!65,#=+(1*<'G'()'3%!3!1'"+'(<*3+#:(H&)$#+1#+'9>(K%'8+;+)'#='(H&!65,%5(,!@#!1$G! )'(H!<!#A(3'#:'&!)*>

L',(1*M$=I(';$(H&)$#+1#+'9!(<*3+$("+$(=$3%(5M*1'"$ 6!(!#)*3)#)'"+'>

;"(#)(,-.,/*#$/((0+.*

5/,5.-$.(/*B),"'.B0*

0(D/(0)-00*<)(),/#$()7

!"#$"%&'%*(#)*""+,'(-...

B.(G$"$&'#=+C

!"#$"%&'%*(#)*""+,'(./

!"#$"%&'%*(#)*""+,'(-...

B..(G$"$&'#=+C

!"#$"%&'%*(#)*""+,'(-...

B...(G$"$&'#=+C

!"#$"%&'%*(#)*""+,'(-...

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5–6




n *E*7.+0*!%"!:F*!8*5/,5.-$.()*+"(#)(,-.,/*#$/((0+.*+-$)%(01#0.G

!"#$%&'()*+(,-.*#%(/0)1+&%(20#3%#"/+".*(314##$2+(2/1%5#$63$+

7(0!031050380)#4(9*45/0):20*+#4(1(0!031+;($(/%20<=$#0*+#4>(

?0#3%#"/+"4(0!031050380)#%(5/0):2:&%(!$6(1(0!031+(@:)12$%,0A(2"./%(

&%!"(=0,+"%(*(*$%@%(314##$2.*(2/1%5#$63$+A(&+2(/.*#$%B($##%(=$+-2+>(

C"+54(5/0):23&$(20#3%#"/+".*(314##$2+(

0!031050380)#%,0(!'(#+!"65:&'3%D(

l E+#4(314##$2(2/1%5#$63$+(&%!"(0))1$%@+#4

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$(:<$%!131+(*(!12@+#438(J0@2+38>

+ !"#$%"&'()#*+,-./#+01234(-/+#2#.5(6(-7#$&$*(0+(8#+0('5"*(-/+

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(0-,*-12#13&,!3--"(#&(2!*+-"4,"#5&6& #7.!*8&,!49,"&1*:0&20! !"#$;<

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5–7




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1(80)0*@$(20<./%2(*!14!"2$38(!:=!"+#3&$A(501+(50B')+#4<(

314##$2$%<(2/1%5#$63$+F

l (G14##$2(2/1%5#$63$+(5/1%2!1"+-3+(!$6(*(*4!:!10#4(!:=@$<+34&#$%(

5/0!1%2($(:<$%!131+(*(!12@+#438(J0@2+38>

E0(5$%/*!1438(20#3%#"/+".*(/%20<=$#0*+#%,0(314##$2+(O

9"1*>(314##$2.*(5$%/*!1%&(,%#%/+3&$;()0)+*+#0(+@=:<$#6A(=$+-20(

@:)12$%(1#+&):&'3%(!$6(*(0!031:A(&+20(!"+=$@$1+"0/(314##$2+(O

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20#3%#"/+".*(/%20<=$#0*+#%,0(314##$2+(O >(R(5/145+)2:(0=%3#$%(

5/0):20*+#438(/%20<=$#0*+#438(20#3%#"/+".*(314##$2+(

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5/0):2:&'3%(50B')+#%(314##$2$(2/1%5#$63$+>

R(5/03%!$%(0314!131+#$+(314##$2.*(2/1%5#$63$+(!"0!:&%(!$6(<4!$%(

5/1%3$*3$+-+A(2"./%(!'(#+!"65#$%(:!:*+#%>(E0!"65#4(20#3%#"/+"

(,%#%/+3&$(&%!"(5/0):20*+#4(=%1(:B43$+(=$+-%2(1(0!031+(@:)12$%,0()0(

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2"./%(1+!"65:&%(<4!$%(5/1%3$*3$+-0(*(5/03%!$%(0314!131+#$+>(

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05$%2$(1(0M/0)2+(@%31%#$+(8%<0J@$$>(f%M@$(<+!1(*'"5@$*0M3$A

314(314##$2(=4-(5/1%380*4*+#4(*%(*-+M3$*438(*+/:#2+38A(

5–14




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l I/1%380*:&(!5/16"(50"/1%=#4()0(50)+*+#$+(20#3%#"/+":(314##$2+(

9!"/142+*2$A($,-4($"5>;(*(314!"4<($(=%15$%31#4<(<$%&!3:A(

#$%)0!"65#4<()@+()1$%3$>(

l R4/1:3+&(1:B4"%($,-4($(!"/142+*2$A(#+(2"./438(1#+&):&'(!$6(M@+)4(

2/*$A()0(!5%3&+@#%,0(50&%<#$2+A(2"./4(#+@%B4(0))+H()0(:"4@$1+3&$

*(50/+)#$(@:=(!15$"+@:>(^$%(*0@#0(,0(*4/1:3$H()0(M<$%"#$2+(5/14(

)0<:>(I/1%!"/1%,+&(/+)(0M/0)2+(@%31%#$+(8%<0J@$$()0"431'3438(

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n *H.+0*-"'$.7*0*7.+8*'.5+1*+"(#)(,-.,9*#$/((0+.*%"'.5.K*'$0)#+9G***

f%M@$()1$%320(/%+@$1:&%(5/0,/+<(@%31%#$+()0<0*%,0A(0"/14<+!1

0)(0M/0)2+(@%31%#$+(8%<0J@$$()02-+)#%($#!"/:23&%()0"431'3%D

l @%31%#$+(2/*+*$%PD

7(/0)1+&:(20#3%#"/+":(314##$2+A(&+2$%,0("/1%=+(:B4HA

7(*$%@20M3$()+*2$A

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l 1+50=$%,+#$+(2/*+*$%#$0<(95/0J@+2"42$;D

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7(*$%@20M3$()+*2$A

7( 316!"0"@$*0M3$(50)+*+#$+(314##$2+>

5–15




]%(1+@%3%#$+(=6)'(+2":+@$10*+#%(*(1+@%B#0M3$(0)("%,0A(&+2()0=/1%(

)1$%320(/%+,:&%(#+(50)+#'()+*26($(#+(316!"0"@$*0MH(50)+PA

0/+1(*(<$+/6A(&+2()1$%320(/0M#$%>

^+@%B4(1+*!1%(5/1%!"/1%,+H(1+@%3%P(0M/0)2+(@%31%#$+(8%<0J@$$

0/+1(1+)+*+H(54"+#$+(*(/+1$%(*'"5@$*0M3$>(S:!$!1D

l 50)+*+H(20#3%#"/+"(314##$2+(1,0)#$%(1(8+/<0#0,/+<%<(@:=(

#+"438<$+!"(50(*4!"'5$%#$:(2/*+*$%#$+F

l :1:5%-#$+H(T)1$%##$31%2(2/*+*$%PU(50(2+B)4<(50)+#$:(

314##$2+F

l 1+*!1%(20#"+2"0*+H(!$6(1%(!*0$<(0M/0)2$%<(@%31%#$+(8%<0J@$$(

@:=(1+=/+H()1$%320()0(!15$"+@+A(&%M@$(5$%/*!1%(50)+#$%(314##$2+(

#$%()+-0(031%2$*+#%,0(%W%2":>

!"#!$%&'"()'$*!$&(+',-.-/0 Najpierw czynnik!

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0)(0M/0)2+(@%31%#$+(8%<0J@$$(2+/"6(9@%,$"4<+3&6;(380/%,0

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l &+2$(/0)1+&(20#3%#"/+":(314##$2+(1+!"0!0*+HF

l &+2'()+*26(50)+H(*(5/145+)2:(*4!"'5$%#$+(/.B#438(

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&+2()1$%320(/0M#$%A(+=4()05+!0*+H(!$6()0(&%,0(1<$%#$+&'3438(!$6(

50"/1%=>

L9!0!$*$.5!$)*B0)K*%-$/*!"F0)*+.-,1*#&"-)<"*

(.*5/%.')+M*<'/F/*%",-$)F(.*F/N.*(.<N.*%"B"#O*

5–16




n *E*7.+0*!%"!:F*3)+.-$)*"F30#$.78*'.5+1*#$/((0+.*+-$)%(01#0.G**

_%2+/1(1(0M/0)2+(@%31%#$+(8%<0J@$$()%34):&%A(&+2$%()+*2$(314##$2+(

#+@%B4(!"0!0*+H(:()+#%,0(380/%,0(*(/.B#438(!4":+3&+38>(^+()%341&6

0()+*3%(314##$2+(*5-4*+D

l I#.#&,"#$#<(R(<$+/6(&+2()1$%320(/0M#$%A(*1/+!"+("%B($@0MH(

20#3%#"/+":(314##$2+A(50"/1%=#%,0()0(@%31%#$+(2/*+*$%#$+F

l J0 !#8&(26#6"*-"#<& <(50*+B#$%&!1%(2/*+*$%#$%A("4<(*$63%&(

20#3%#"/+":(314##$2+(50"/1%=+A(+=4(&%(*4@%314HF

l '#(&.!3)(0&20!+0,!410&7*,!*-"*<&?/*+*$%#$%A(2"./%(5010!"+*$0#0(

=%1(@%31%#$+(#+(2$@2+(,0)1$#A(*4<+,+(*$62!1%&A(+(31+!%<(#+*%"(

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l K3+&A*B0C7""<&E+*20*+#$%(0=@$31+(!$6($#+31%&()@+(#$%)0=0/:(

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L)7",!#-"*& #6(06#-"#<(]+=%@%D(Y($(j(502+1:&'($@0MH(314##$2+A(2"./'(

@%2+/1(1*42@%(!"0!:&%(5/14(/.B#438(/0)1+&+38(2/*+*$%P>(E+*26(

*4/+B0#0(*(&%)#0!"2+38(<$6)14#+/0)0*438(9&><>;(20#3%#"/+":

#+(2$@0,/+<(<+!4(3$+-+>(

5–17




?"'$.7*+-5.50)(0.

>"D8'.(/*%"$0"B*#$/((0+.*P@@@ 5)*+-50 2Q*("-B/6

>"#$8,+"5.*'.5+.*#$/((0+.*P@@@25*B01'$/(.-"'"5/#&*7)'("!,)+*(.*+03"<-.B*B.!/*#0.N.6

Tabela 2

>-"%"("5.()*'.5+0*#$/((0+.*P@@@*$.%"F0)<.78#)*+-5.50)(09*39F*%"$5.3.78#)*%"5!,-$/B.K*7)*5*-:D(/#&*"+"30#$("R#0.#&

ST=SU*7CBCV+<

WT=WU*7CBCV+<

XU=UT*7CBCV+<

WT=WU*7CBCV+<

WT=XTQ

YT=UTQ*

ZT=STTQ

YT=UTQ*

!"#!"$%!"&%'#(#!")!"&#"#)*+',)"-l !"#$%&'#()*' &+# ,'-.'$() ,'+,/'0123%1)4

l *. )5%&'67 .(.6'"'%.$)4l ,(7"&0,89!&'$12'67 1.0.!"4'l 67 ) 1#%1#' '8)01#',$(%#84' 6(:7#'%1#'7#);,8#'%)'<=)!&+>

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l 67 ) 1#%1#' # %2(7"%# './72/1#'6)76,4'82"&6)4';)7-@)4

l *7.A+)6(&6)'*.',7)"1#4';-"1#'%1#' &$(9*1@.'67 ) 1#%1#>

5'#(#!")!"&,(6'(0(789"&0:9!3-l *. )5%&',7)"';@. &4l ")/1#;'!B17,7;1!"%&'C.*7:!"'")/1#;: '-#%(&$(&!"%&!BD4

l 67 ) 1#%1#'*.'*. )5%&0',7)"1#4l 67 ) 1#%1#' './72/1#'/7",!B)>

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* E'*7"&*)-6,',$, )%1)'"2/)'%)+#5&'*7"#"'61+6)'-%1'*.-) )F'<=)!&+'8)6.'-.-)(#6'-.'!"&%%16)'

67"#*%12!1)>'

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'&EA&)3&JEA1&

5–18



Leczenie czynnikiem %',"?)!*9!(5

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Tabela 3

.'/?/)/#()"&2(#%!&9,:))!%(&BC&,(?/=!"6(789"&%'#(#!")!3&$3=&?/,#($(789"&?/#<+',:A(G&7"&#&'H0):9E&/%/$!9,)/I9!(9E

JKLMK&7NANO%6

KPLQP&7NANO%6

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l *. )5%&'67 .(.6'"'%.$)4l ,(7"&0,89!&'$12'67 1.0.!"4'l 67 ) 1#%1#' '8)01#',$(%#84' 6(:7#'%1#'7#);,8#'%)'<=)!&+>

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l 67 ) 1#%1#' # %2(7"%# './72/1#'6)76,4'82"&6)4';)7-@)4

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5–19




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5–20




n &V(%&2F36/&?/&?/2()!3&+'#(&2,!(F()!"&9,:))!%(&%',"?)!*9!(W&&&

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n & (&!$"&=",?!"9,)"&<8&%/)9")+'(+:&9,:))!%(&%',"?)!*9!(W

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Leczenie czynnikiem !#$%&'(&)idn.org.pl/hemofilia/rozdzial5.pdf · 5–8 Leczenie czynnikiem 5 +!"#$%&$'()*+)$,-.+