sOvrE ceNG uoa xe ugr cuu Ncuia vrpr r\AM
BENH VTNX NIII TRUI{G TIOiVC DQc l$p - TU do - H4nh phric
Hd NOi, nsry // thdng l0 ndm 2013
QUynr DINH
\Lt---- V/v Ban hinh PhSc dO chin do6n, di6u tri bQnh Hemophilia
GIAM DOC BENH VIEN NHI TRUI.{G UOI\G
Cdn cir Quy6t dinh s6 1 1 1/CP ngdy 1417llg6g cua HQi d6ng Chinh phu vC
viQc thdnh lpp ViQn B6o vQ sirc kho6 tr6 em nay ld Benh viQn Nhi Trung uong;
i,
Cdn cri' Quy6t dinh s6 169312004/QD-BYT ngey 141512004 cira BQ
trudng 89 Y ti5 bu, hdnh Di6u 19 TO chric vd hopt dQng ctia BQnh viQn Nhi
Trung uong;
!
i,
Cdn cri Nghi dinh sd 29120124{D-CP ngay 12/042012 cin Chinh phu v0
.A
viQc tuy6n dung, st'dr;ng vd qu6n ly vi6n chric;
CIn cri Nghi dinh s6 4ll2}12AID-CP ngey O8lO5l2}l2 oiua Chinh phu vd)
I
viQc quy dinh vO vi tri ldm viQc trong dcrn vi sg nghiQp c6ng 16p;
CEn cir y6u cAu kh6m, chta bQnh cua nh6n dAn;
Theo dA ngh! cria 6ng Truong phdng KO hoach t6ng hqp,
QUYET DINH
Di6u 1. Nuy, ban hdnh Phdc d6 chAn do5n, diAu tri bQnh Hemophilia - Bqnh
vien Nhi Trung uong (nhu vdn b6n kdm theo).
Ei6u 2. Quydt dinh c6 hiqu lUc t<O ttr ngey 01 th6ng 10 ndm 201,3.
Di6u 3. C6c 6ng, bd trucrng ph.ong T6 chirc c6n bQ, K6 hoach t6ng hqp, Tdi
chinh kti to6n, c6c khoa phong c6 1i6n:quan chiu tr6ch nhiem thi
hdnh qry6t dinh nay./.
.'ru
DOC
"/
't.,1
a$we v;dro
Noi nhQn: \,-
- Nhu di6u 3; TI\THulrs ufiH
- Luu VT, TCCB.
PHAC DO CHAN OOAX OIUU TRI BENH HEMOPHTLTA
(Kdm theo quytit ai* sa.,lfLllqD - TCCB , nsiry/t/t.th6ns 10 n[m 20t3)
1. DINH NGHiA
Hemophilia ld b6nh r6i lopn d6ng m6u do thi6u m6t trong ba y6u tO AOng m6u (y6u
t6 VIII, IX vd X), bQnh it ew chu y6u ld o tr6 trai 3 - 5 tr6 / 1000 000 so sinh.
2, NGUYON NHAN
BQnh Hemophilia li benh d6t bi6n gen t6ng hqp c6c you t6 VIII, IX, )il , rdt it
trudmg hqp do nguy6n n\en mic ph6i (nhu kh6ng th6 khang y6u t6 Vm, IX )
3. LAM SANG
Bi0u hiQn chinh cua bQnh ld d6ng m6u kdo ddi (m6u kh6 d6ng) g6y ra t.u m6.u vd
ch6y m6u k6o ddi.
Tg m6u :
Tp m6u c6 thO xby ra bbdtkj,noi ndo cua co th6 , nhrmg ddc tnmg nhAt cho bqnh
Hemophilia ld tu m6u 0 khcry ( khdp g6i, , khuyu, bdvai , tuy ch6n d6i khi e khop
"6
h6ng ).Kh,ry sung , dau, han chti vdn dQng, t.u m6u t6i diSn gaty cungkh6p , teo ccr
phia tr6n vd du6i khop )
Tu m6u d co diri, bip chan , co mdng , c6nh tay, cbngtay . Tp m6u cy co d6i chQu dE
rl
nhAm v6i vi6m ruQt thtra.
Chiy m6u k6o dii
Sau ch6n thuong nhu: khi bi dirt tay, chdn, ch6y m6u chAn rdng do nh6 rdng , va
dflp d miQng. Sau ph5u thu4t chdy m6u kh6 cAm m6u c6 th6 g6y ra t.u m6u lcm ho{c
mat
^. mau n?ng.
ChAy m6u c5 th6 x6y ra o ducrng ti6u h6a, dudng ti0t ni6u . Chhy m6u d nao , cr ph6i
co thti g6y tu vong hoflc tdn phtS ndng.
4. CHAN DOAN
4.1. ChAn doin xfc tlinh
- DUa vdo lAm sdng : BAm tim , t.u m6u , ch6y mdu
- DUa vdo tidin su gia dinh
- DUa viro x6t nghiQm :
Bu6c 1 : 36 lugng ti6u cAu binh thucrng
PT binh thucrng
Fibrinogen binh thulng
APTT k6o ddi > 1,5 gi|tri binh thucrng
Buoc 2: y6u t0 VIII (Hemophilia A) ho{c y6u t6 IX gi6m du1i 40 %
(Hemophilia B) hopc y6u tO )(I gihm duli 40%
y6u t6 von Willebrand binh thucrng
- Cdc xdt nghiOm kh6c Mixtest x5'c dinh khang th6 kh6ng y6u t6 VIII hoflc IX.
4.2. ChAn dodn mric d6 b6nh
Mric dd NOng tIQ y6u tO oh Bi6u hiQn chiy m6u
ho4t tinh (tlon vi/ml)
NIng 1%(<0.01) Chiy m6u fu nhi6n kh6ng 1i6n quan
(Chi6m 7Oo ) il5n ch6n thuong, thudng chiy m6u
6 khop vd co
Trung binh 1-s%(0.01-0.0s) ChAy m6u t.u nhi6n hoflc 1i6n quan
(Chi6m rs%) d6n ch6n thuong nho. Chiy m6u
n{ng sau ch6n thucrng, phSu thupt
Nhe s-40% (0.04-0.4) ChAy m6u sau ch6n thucrng lml
(Chi6m 15o/o) ho6c phdu thupt
s. DIEU TRI
5.1. Nguy6n tic chung
- Trong di6u kiQn hiQn nay nguy€n tAc diAu tri ld cAm m6u khi c6 chiy m5u tp m6u
- Chiy m6u c6p n6n dugc ctiiu tr! cdng sdm cdng fit,t}tnnat U sau 2 gid.
- YOu t6 d6ng m6u cAn duoc b6 sung dO c6 th6 dat dugc n0ng dO dt d6 cAm chiy m6u.
5.2. Diiiu tri il4c hiQu
Vi6c diAu tri thay thO php thuQc rat nni0u vdo tinh chht cinc6c ch6 phAm m5u c6 sin vd
thoi gian b6n htry cua y6u tO cl6ng m5u. Thdi gian b5n huy cua y6u tO VIII la 8-12 gid, cua
y6u tO IX la 24 giir. Thdi gian b6n huy c6 the rft ngin ndu bQnh nh6n chtry mtnnhiOu hopc
c6 chAt ric ch6.
CSch tinh li6u y0u tO VIIV IX cAn dua vdo:
- Edi v6i hemophilia A: 1 dcrn vi y6u tO VItVkg th6 trong c6 th6ldm tang rr6rg dQ y6u t6
VIII cria benh nhdn 16n khoing2% .
- DOi vdi hemophilia B: 1 dcrn vf ytiu tO t)Vkg thr5 trqng co thti ndng n6ng dQ y6u t6 IX ctra
bQnh nhnn lOn dugc khoing 1% .
Truydn yOu t6 VIII trong Hemophilia A (Hemophil M, Dried Factor), y6u t6 IX trong
Hemophia B (Bene FIX , Alpha Nine).
-Li6u ,
'x lugng A .A
yeu t6 ddng m6u phq thuQc vio vi tri chhy m5u theo bing
IlemophiliaA Hemophilia B
N6ng d6 Thdi gian Non sd0 Thtri gian
V! tri chfry mfu I
cAn clat (neny) can ilat (ngnv)
(%) (%)
Kh0p 20-30 1-3 c5 th€ k6o 20-30 1-3 co th6
ddi hcrn n6u k6o dii hcyn
chua dd n6u chua dd
Co (tru co d6i 20-30 2-4 c6 th6 k6o 20-30 2-4 c6 thd
ch0u) ddi hcvn n(5u k6o ddi hon
cnua d0 n6u chua dd
Co d6i chQu
..i,
+tan cong 20-40 t-2 20-30 1-2
+duy tri 10-20 3-5, c6 the k6o t0-20 3-5, co th6
ddi hcvn n6u k6o ddi hcrn
cnua dc, ndu chua dd
HQ thdn kinh TtJ
. .A
+ tan cong 50-80 1-3 s0-80 1-3
+ duy tri 30-50 4-7 30-50 4-7
20-40 B-14 (hoac 21 20-40 8-14 (ho4c 21
ngdv) nga.y)
Cd vd ngpc
+ tan c6ng 30-50 1-3 30-50 1-3
+ duy tri I0-20 4-7 t0-20 4-7
XuAt huy6t ti6u h6a
+, tan
'A
cong 30-50 1-3 30-s0 7-3
+ duy tri t0-20 4-7 r0-20 4-7
D6i m6u 20-40 3-5 t5-30 3-5
VCt thucyng sdu 20-4Q 5-7 15-30 5-7
Phflu thuAt
* tru6c 60-80 50-70
t sau 30-40 1-3 30-40 1-3
20-30 4-6 20-30 4-6
l0-20 7 -14 10-20 7-14
C6c ch6 phAm diOu tri khdc:
r Tira l?nh, m6i dcrn vi tua lanh chria B0 - 100 don vf ydu t6 VIIIo/100m1
Plasma tuoi.
sau khi ny ( truy6n cho hemophilia B )
, ,. .Plasma lalAnh
C6c thudc c6m m6u hd tro ---+ di6u tri khi c6 ch6t irc ch6.
5.3. DiAu tri Hemophilia c6 chAt ri'c ch6
Kho6ng 10 - 15% hemophilia A vd l-3% hemophilia B c6 chAt ric ch6
Khi c6 ch6y m5u diOu tri nhu sau:
- NOu n6ng dQ ch6t ric chO < 5 BU, diAu tri 1i6u cao ydu t6 c6 d[c
- NOu n6ng dQ chAt ric ch6 > 5 BU di6u tri bang ydu t6 ducrng tit: y6u tO VtIa hoflc phric
hqp prothrombin cO ddc APCC (FEIBA, Autoplex).
a-
+ VIIa 9OIII/kg m5i S gio tdi khi drmg ch6y m6u . Trong ph6u thuQt 16n hodc ch6y m5u
nflng c6 th6 ti6m 9OIII/Kg tu 2-4 giiil llAn trong 6-7 ngdy dAu.
+ APCC (FEIBA) 50 -100 UI/kg ,m5i 6 gio dOn khi drmg chiry m6u
Di6u tri dung npp miSn dich trfti n6ng dQ chAt ric ch6 dang hj, gi6m xu6ng
N6u ndng tlQ ch0t irc chO > 15BU cAn di6u tribang t6ch huyi5t tu*g, ti6p theo d6 ld di6u
.x
tri dung npp mi6n dich.
YCu t6 VIII 50UI lKg ,3 lAn / 1 tuAn
Chri y: 50% benh nhAn hemophilia B c6 phin utrg di rmg n{ng khi dimg yOu t6 IX c6 ddc,
n6n 1 O -20lAn di0u tri ddu ti6n ph6i dugc ti6n hnnh cr bQnh viQn
5.4. Cic thuiic didu tri cAm mfu h6 trq
- T6c nhdn fc chi5 fiOu sgi huy6t: Tranexamic acid vd EACA (epsilon amino caproic acid).
C6 tdc dung t6t trong di6u tri ch6y m6u ni6m mpc: chAy m6u ch6n rin, xu6t huy6t ti6u h6a.
Tuy nhi6n cAn luu 1i kh6ng dirng trong trudng hqp d6i m6u vi cuc m6u ddng kh6ng ti6u c6
th6 gdy tdc 6ng thQn, niQu quAn.
Tranexarnic acid (Transamin) 25mglkg cAn nflng mdl O-A gio trong 5- 10 ngdy
-Desmopressin (cdn ggi te DDAVP) c6 t5c dr,rng t6t tr6n bQnh nhdn hemophilia A mric dQ
nhg vd m6t vdi th6 cua bQnh von Willebrand.
LiAu lucrng: 0.3-0.4microgam/ kg th6 trgng pha truydn tinh mach chpm
- Corticoid: co th6 dung I dqt corticoid nghnngdy (<7 ngdy) trong trudng hqp sau khi chAy
mdu khdp dd cAm nhung cdn dau nhi6u do phan ring vi6m. Li6u lmglkgth6 trong.
6. DtI PHONG CHAY MAU
- Can h6t sfc tranh ch6n thuong.
- Tr5nh dung c5c thu6c 6nh huong d6n chric ndng ti6u cAu, ddc bi6t ld acetyisalicylic acid
(Aspirin). D0 giam dau cho bQnh nhAn co th6 dung paracetamol ho{c paracetamol k6t hgp
codein.
- Tr6nh ti6m bip, chAm crlu.
-,1
- Ti0m vdc xin d6y dir, dpc biQt ti6m phdng vi6m gan virut B vd A (kh6ng drxrg ducnrg ti6m
b[p mi chuy6n sang ti6m dudi da)
;"
- Tap th6 duc hang ngdy dti tang th6 lpc, tdng sric m?nh co b6p, girip khop hoat d6ng t6t
hcrn vh giup han ch6 chiy m6u co khcrp. Tr6nh c6c hoat dQng th6 thao mang tinh chAt dOi
kh6ng nhu boxing, ddb6ng, d6u vpt. Ndn tAp boi, di b0 vi di xe d4p.
