BNH L NG MU

HUYT TNG
I.Phan gii thieu
II.Benh ly ong mau huyet tng di truyen
III. Benh ly ong mau huyet tng mac phai
TS Huynh Ngha
B mon Huyet hoc, H Y D .

M AU
I. Benh ly MHT di truyen : Do thieu 1 yeu to
- Benh Hemophie A (VIII) , B (IX), Von-wiilerbrand.
- t gap: Benh Rosenthal (XI), Heagement ( XII)
- Benh thieu hut nhieu yeu to MHT di truyen
II. Benh ly MHT mac phai: Do thieu nhieu yeu to
- Thieu Vitamin K
- Suy gan
- Khang ong lu hanh
- ong mau noi mach lan toa

SINH LY ONG VA CAM MAU

1.Sinh ly ong cam mau la s thang bang ky dieu 2
qua trnh :
ong mau

Chay mau

2.bao gom 3 giai oan :

* Giai oan cam mau s khi

* Giai oan ong mau huyet tng
* Giai oan Tieu si huyet

NG ONG MAU NOI SINH

ng mu ni sinh
XII,
XI
IX, VIII
Ca+2

Phospholipid
and Ca+2

ng mu chung
FX,FV,Ca+2
II

IIa
I

Ia

NG ONG MAU NGOAI SINH

ng mu ngai sinh
Tissuefactor
Ca+2
F VII

ng mu chung

FX,FV,Ca+2
II

IIa
I

Ia

Phospholipid
and Ca+2

NG ONG MAU CHUNG

II

Thrombine

XIII

fibrinogen

2 peptid A, B

Mono fibrin

XIIIa

Fibrin soluble
(FS)

Trung hp

Ca+2

Fibrin
insoluble (FI)

S TNG TAC CUA 2 NG ONG MAU

XII
XI
VII

IX
VIIa

VIII

V
II

THROMBIN

S KHUYEC AI CUA HIEN TNG ONG MAU

VIII
VIIa

VIIIa

V
Va

Thrombin

CAC YEU TO ONG MAU B TIEU THU TRONG

QUA TRNH ONG MAU

I-II-V-VII
VIII-IX-X
XI-XII
Tieu cau

VII
IX

I
II

V
X

XI
XII

VIII
Tieu cau

MAU TOAN PHAN

HUYET THANH

CUC MAU ONG

GIAI OAN TIEU SI HUYET

Yeu to to chc
XIIa
Plasminogen

Urokinase

fibrin
Plasmin
PDF
Anti-plasmin
( Manh X,) Y, D (
( X,Y,D,E
D-dimer , E)

Streptokinase
Kch hat
c ch

Cc xt nghim kho st
MHT

Ni sinh :
aPTT ( activity Partial Thromboplastin Time)
aPTT mix ( hn hp)

Ngai sinh :
PT ( Prothrombin )
PT mix ( hn hp)

ng mu chung :
nh lng Fibrinogen
Thrombin times ( TT)
nh lng D-Dimer

Activated partial thromboplastin time (aPTT)

Temps de Cephalin Kaolin ( TCK)

Intrinsic pathway
Kaolin,
celite,
ellagic acid

FXII,
FXI
FIX, FVIII
Ca+2

Phospholipid
and Ca+ 2

cephalin

Common pathway
FX, FV, Ca+
II

IIa
I

Ia

BT : 30-50 giay . Bat thng > chng 10 giay

1. BT : 12-14, Bat thng > chng 2

2. Taux de prothrombin BT : 70-100% Bat thng< 60%
3. INR ( International Normalized ratio) : BT <1.2

Thrombin time

Thrombin
ngai sinh

X, V, Pf3
Final stage

II

IIa
I

Ia

* BT: 18-20, Bat thng > chng 7

* Y ngha : - Giam Fibrinogen < 1g/l

- Tang Fibrinogen > 6g/l

- RLCT PT Fibrinogen
- Khang ong chong Thrombin ( Heparin)

NH LNG FIBRINOGEN
1.Muc ch : nh lng yeu to I, tong hp GAN

2.Ky thuat :

- KT Ket tua nhiet (*)

- KT o lng trong lng ( chuan nhat)
- KT o thi gian

3.Ket qua :

- BT : 2-4g/l
- Giam < 1,5g/l - Tang >6g/l

4. Y ngha :
- HC giam Fibrinogen :
+ Bam sinh
+ Mac phai : Suy gan, DIC, TSH tien phat, Thuoc
- HC tang Fibrinogen : viem nhiem man tnh
- HC RL cau truc phan t Fibrinogen di truyen hoac mac phai

BENH LY ONG MAU HUYET TNG DI TRUYEN

NHOM BENH HEMOPHILE: H.A (VIII), H.B( IX)
1.Di truyen : NST gii tnh X, mang tnh lan
2.Trieu chng lam sang:
- Tnh trang xuat huyet t le thuan vi nong o thieu hut
- Cac TC thng gap :
+ XH Khp : Khp goi, khuy, mac ca, hong, co tay.
Hnh thai cap---> Viem bao hoat dch--> VK man --> mat chc
nang tan tat : ganh nang gia nh va xa hoi

elbow
hip

knee
ankle

+ Chay mau trong c

- C ui

- C cang chan

- C thang ( mau tu, mat mau, nhiem trung)

+ Xuat huyet than kinh trung ng : t le t vong cao
+ Chay mau sau phuc mac- thanh quan
+ Tieu mau : cn au quan than, than trng nc
+Chay mau keo dai trong va sau phau thuat :
- Sau nho rang
- Sau phau thuat chnh hnh

3.Xet nghiem :
- Thi gian mau chay : BT ( TM tot)
- PT : BT

- Tcau : SL & CL : BT

- aPTT ( > chng 10)

- aPTT hon hp : ngan lai bnh thng

Pbn + Pbt Phh Xn aPTT aPTT hh
Benh nhan

Hon hp

Benh ly
Thieu hut cac YTM
Khang ong lu
hanh

- nh lng yeu to VIII va IX: nh benh, mc o va

tien lng benh

4. Phan loai mc o Hemophilie : [ VIII] , [IX]

- Nang : 1%
- Nhe : 5-30%

- TB : 2-5%
- Khong TCLS 30%

5.Chan oan phan biet :

- Thieu XI ( Rosenthal- H.C) , thieu XII ( Heagement)
- He.A Benh von-Willerbrand, KLH chong VIII, HCDT V+VIII
- He.B thieu VitK, suy gan,KLH chong IX

6.ieu tr :
Tong quat : -Khong uong Aspirin va thuoc chong KDTC, khong TB
-ieu tr thay the YTM thch hp
-Hng dan su dung thuoc ong kho
-Lap chng trnh trc phau thuat

 Hemophilie A :
Demopresson (DDAVP): Phong thch VIII& IX t ni d tr, [VIII]
- C : nhe va TB
- Lieu : 0,3ug/kg TM ( 30, 8gii), 300ug/lan xt mui
- Td phu: o mat, au ngc, nhp tim nhanh
Yeu to VIII ket tua lanh hoac ong kho :
F.VIII KTL : TT: 30-50ml, [VIII] : 3-5v/ml , TB : 150v/ tui
F.VIII ong kho : 1000v- 10.000 v/ml
C : Nang, nhe hoac TB khi can thiep p/thuat chay mau nang
Lieu : Px 70 x (1-0,4) x [ Nong o muon at en] = So /v
thi gian ban huy = 12 gi , x 2 lan / ngay
Td phu : lay lan benh virus B,C, HIV. KLH. D ng.

 Hemophilie B :
PCC ( Prothrombin Complex Concentrate) II,VII,IX,X: t dung

HTL/HT ti : Nhe/ TB , 10-25ml/kg/ngay .VGB,C,HIV

F.IX on kho : Nang , TM cham , T1/2 = 24 gi

ieu tr thch hp cac trng hp chay mau:

XHK : - Nhe : Chm a, giam au, co nh khp. TB/ Nang :
TThay the , [ 30-50%] x 5-7 ngay.Neu kem ap ng : KLH hoac
NT bao hoac dch : khang viem + khang sinh

Mau tu trong c : >[ 50%] x 5-7ngay

XHN-MN, XHTQ : [ 100%] x 7-10ngay
Tieu mau : Ngh ngi, tang dch truyen,>[30%], CC: Chong TSH
Phau thuat : [80-100%] trc va sau PTx 7 ngay
Amino caproid acid ( 4gr/6gi) , Transamin 0,25mg/kgx3 ( PTHM)

 NHOM BENH RLM DI TRUYEN KHAC:

ac iem : - hiem gap

- DT NST , mang tnh lan

- RL giam Protein chuyen ch, giam YTM.
XII ( Haegement) : khong co TCLS, kem thuyen tac mach
XI ( Rosenthal) = Hemophilie C : LS giong nhom benh Hemophile
Benh von-Willerbrand : NSTmang tnh troi, TCLS : HC xuat huyet
dava niem mac, XN : TS , TCK , TCKhh , TQ , [VIII]<30%, [VIIIvW] <30%, SLTC , o Ngng tap TC : . T: Truyen YTVIII kt lanh,
truyen TC ( chay mau nang)
XIII : TCLS : XHK, chay mau sau PT,rong kinh . XN : TS,TQ,TCK ,
Nghiem phap 5Ure M (+)
V : TCLS: U mau trong c, rong kinh, CM cam .XN : TS , TCK ,
TQ , TT , Fibrinogen , SLTC :

BENH ROI LOAN ONG MAU HUYET TNG MAC PHAI

Thieu Vitamin K san sinh cac Protein ong mau bat thng:

Nguon goc : Thc vat(K1-phylloquinon),ong vat(K2Farnoquinon), ngi cung cap K1( thc an) ,VK ng ruot
tong hp (K2)

Hap thu : mang ruot sau hoa tan acid beo ( muoi mat), gan
albumin ve gan.Tai gan c tong hp gan them Gamma
carboxy, mi co them bam len phospholipid TC mang hoat
tnh ong mau . Khi thieu VitK, Gan tao ra cac YTM cha
hoan chnh va khong co hoat tnh

Thieu Vitamin K san sinh cac

Protein ong mau bat thng:
Nguyen nhan thieu hut :
Thieu cung cap
Hoi chng kem hap thu
Qua lieu thuoc chong Vitamin K
Thieu Vitamin K tren tre s sinh

Trieu chng :
Tre s sinh : Chay mau muon : XHTH, XHNMN, tieu mau
Tre em va ngi ln : CM mui, li rang, rong
kinh, bu mau

nh benh :
Nhe : PT , aPTT , T1/2 VII:4-6 gi, nh lng YT VII
gim
Nang: PT , aPTT , YT II, VII, IX, X gim F.V
Tm nguyen nhan : Test Kohler
ieu tr :

Tre em va ngi ln : Vit K1 : 10mg TM, TDD, nang : HTL

Tre s sinh : Phong nga sau sinh 2mg TB , uong x 3 ngay,
nang HT ti ong lanh 15-20ml/kg/ngay x 3 ngay
TM co nguy c shock phan ve, bu mau : TDD hoac TB

Suy gan giam tong hp

cac yeu to ong mau
Gan: la ni tong hp cac YTM
YTM phu thuoc Vit K : II, VII, IX, X
YT I, V, XIII
- YT chong TSH : PlasminogenAnti Plasmin
YTC M : Anti Thrombin III, ProteinS, ProteinC

Trong suy gan: Chay mau do giam YTM , do

giam YT van chuyen ( albumin), do h hai TM
( tham thau, v TM trng thc quan)

Suy gan giam tong hp

cac yeu to ong mau
Nguy c DIC : do hoai t TB gan ( Thromboplastin),
do kch hoat cac YTM, do giam cac YTC M
Lam sang : co hai hnh thc CM do suy gan
Suy gan va : t co TC, phat hien tnh c, cac
YTM giam sm VII, X ( PB thieu Vit K ), tm
nguyen nhan suy gan,
Suy gan nang :
- Tat ca YTM eu giam, Gan khong thanh loc
c YTM kch

Suy gan giam tong hp

cac yeu to ong mau

Hoat DIC man. Tang hoat tnh TSH ,

SLTC giam do cng lach roi loan
sinh hoc phc tap va kho bien luan .
nh benh :

PT , aPTT , I , TT , cac YTM giam

YT V va Anti Thrombin III : tien lng
Ethanol (+). FDP , DIC man hoac cap
kem theo

Suy gan giam tong hp

cac yeu to ong mau

ieu tr :
+ Nguon goc gay suy gan ( neu co the c)
+ Truyen HT ti L khi co chay mau : 2030ml/kg/ngay
+ Truyen TC khi SLTC< 20.000/mm3.
+ PPSB khong s dung v tang nguy c DIC,
Vitamin K khong hieu qua
+ DIC : truyen HTL, tieu cau , Anti
Thrombin III, Heparin khong C

Khang ong lu hanh

/n : S xuat hien KT chong lai cac YTM- hay can
tr mot giai oan ong mau

o
o
o
o

KLH trong benh RLM di truyen:

D KT do truyen YTM nhieu lan, thng gap trong benh

He.A (15%) He.B ( 2.6%).t gap hn YTV, VII, XII, XIII
Thng benh nhan co TC nang ne hn, khong ap ng vi
T cu. XN hon hp khong rut ngan c
T le t vong cao, t ap ng corticoit va thuoc C mien
dch
ieu tr truyen tang gap oi lieu YTM , Truyen YTVIIa

Khang ong lu hanh

KLH mac phai :

KT chong phospholipid TC

Thng gap n >nam, tuoi thng gap > 35 tuoi

Chay mau : giam TC, KLH c che Phospholipid
Huyet khoi tnh mach : chi, chau, TM ca gan

Cc khng th t nhin chng mt yu t ng mu ,

bnh l Hemophlia mc phi :

Tn sut hng nm khong 1.5 /1000.000 ngi v thng nh

hng trn ngi gi vi tui trung bnh khong 75 tui , t l nam
v n tng ng, ngoi tr nhm bnh nhn tr tui n cao hn
i km vi thai k.

Cc triu chng chy mu bnh nhn

Hemophilia mc phi
V tr chy mu

Tn sut chy mu (%)

Yu cu tr liu

Di da lan rng

81

23

Trong c

45

32

Tiu ha/ bng

23

14

Sinh dc tit niu

18

Sau phc mc/ ngc

Sau m

11

Khp

Xut huyt no

Khc

23

Khng xut huyt

Khng p dng

T vong

Khng d liu

ONG MAU NOI MACH LAN TOA

1. nh ngha :
2. C che benh sinh
3. Bieu hien lam sang:
4. Nguyen nhan DIC
5. ieu tr

NH NGHA DIC
1, ong mau : s mat can bang gia he ong mau va
tieu si huyet, dan e tang ieu thu cac yeu to ong
mau, va tao nhieu huyet khoi trong long mach
2, GPB : Nhieu cuc huyet khoi trong long mach gay
tac nghen , huy hoai mach mau va ton thng a c
quan

C CHE BENH SINH

TON THNG MACH MAU

HUY HOAI TO CHC

Yeu to ung cham

Thromboplastin tissue

XII
XI
IX

Pf3 + Ca++

VIII

VII

X
II
I

v
Thrombin

XIII
Fibrin S

Ca++

Fibrin I

S TNG TAC CUA 2 NG ONG MAU

XII
XI
VII

IX
VIIa

VIII

V
II

THROMBIN

S KHUYEC AI CUA HIEN TNG ONG MAU

VIII
VIIa

VIIIa

V
Va

Thrombin

CAC YEU TO ONG MAU B TIEU THU

I-II-V-VII
VIII-IX-X
XI-XII
Tieu cau

VII
IX

I
II

V
X

XI
XII

VIII
Tieu cau

MAU TOAN PHAN

HUYET THANH

CUC MAU ONG

GIAI OAN TIEU SI HUYET

XIIa

Yeu to to chc

Plasminogen
Plasmin
Anti-plasmin

fibrin

PDF
( X,Y,D,E )
Fibrinogen

Vong noi mo
Gan, Lach
PgDF

Giam Tr.hp Fibrin

Giam ngng tap Tieu cau

TRIEU CHNG LAM SANG

1, Benh canh lam sang chung khi phat DIC
2. Hoi chng chay mau nhieu ni : da, niem mac, c
quan noi tang.

3. Hoi chng tac mach :

- Nao : l m, lu lan , la het .
- Than : Thieu nieu, vo nieu
- Chi : au, sng to, nong

Tieu chuan chan oan DIC

Colmann 1972:
3 TC chnh : Tieu cau giam, PT dai, Fgen giam
KL DIC
2 TC chnh (+) va 1 TC phu (+) DIC
1 TC chnh (+) loai DIC

TC phu : Thi gian thrombin

Np Ethanol ,
Np Von-Kaullar

Ngay nay: Tieu cau, PT, Fibrinogen va

D-dimer

DIC theo WHO

S lng tiu
cu
(x 109/L )

0 im

1 im,

100

<100

D-dimer hoc Khng

FDPs
tng
Prothrombin <3 giy
times
Fibrinogen 1 g / L
Kt qu

2 im
<50

Tng nh
> 3 <6

3 im

Tng
mnh

>6

<1g/L

5 : DIC

< 5 : t ngh DIC

Thang im chn on DIC ca Nht Bn

Tnh trng bnh nhn

im s

H thng p ng vim
- >3
- 0-2

1
0

S lng tiu cu (x 109/L )

- <80 hoc gim 50% trong vng 24 gi
- > 80 v <120 hoc gim 30% trong vng 24 gi
- > 120

3
1
0

Thi gian Prothrombin (PT)

- > 1,2
- < 1,2

1
0

Fibrinogen / FDP (mg/ L)

- > 25
- > 10 v <25
- <10

3
1
0

Chn on DIC

>4

NGUYEN NHAN DIC

Noi khoa :

Ngoai khoa :

San khoa :

Phoi hp

Nhiem trung huyet

Sot ret ac tnh
Benh ly noi khoa giai oan cuoi
Bach cau cap ( M3, va SL > 50.000/mm3)..

a chan thng
Bong o III, > 30% dien tch c the
Thai chet lu
Nhau tien ao

* Nhiem oc thai nghen

* Nhau bong non.

IEU TR

ieu tr benh goc khi phat DIC

ieu tr benh nen
Giai quyet c thanh cong : 50% -75%

ieu tr chong chay mau :

Truyen tieu cau am ac
Truyen huyet tng ti ong lanh
Truyen yeu to VIII ket tua lanh

Hoi sc chong choang, chong suy ho hap

ieu chnh roi loan ien giai va dinh dng
S dung Heparin