Benh Dmht ( Bai Giang)

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

XII, XI

IX, VIII

Ca+2

FX,FV,Ca+2

II IIa I Ia

ng mu ni sinh

Phospholipid and Ca+2

ng mu chung

NG ONG MAU NOI SINH

FX,FV,Ca+2

II IIa I Ia

ng mu ngai sinh Tissue- factor Ca+2 F VII

ng mu chung

NG ONG MAU NGOAI SINH

Phospholipid and Ca+2

NG ONG MAU CHUNG

II Thrombine

fibrinogen

Mono fibrin Fibrin

insoluble (FI)

Fibrin soluble

(FS)

Trung hp

2 peptid A, B

XIII XIIIa

Ca+2

S TNG TAC CUA 2 NG ONG MAU

XII

XI

IX

X V

II

THROMBIN

VII

VIIa VIII

V

Va

Thrombin

VIIa

VIII

VIIIa

S KHUYEC AI CUA HIEN TNG ONG MAU

I-II-V-VII

VIII-IX-X

XI-XII

Tieu cau

I

II

V

VIII

Tieu cau

VII

IX

X

XI

XII

MAU TOAN PHAN HUYET THANH CUC MAU ONG

CAC YEU TO ONG MAU B TIEU THU TRONG

QUA TRNH ONG MAU

GIAI OAN TIEU SI HUYET

Plasminogen Plasmin

fibrin

PDF

( X,Y,D,E )

XIIa

Yeu to to chc

Urokinase

Streptokinase

Anti-plasmin

Kch hat

c ch

( Manh X, Y, D ( D-dimer , E)

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 partialActivated partial thromboplastinthromboplastin timetime (aPTT)(aPTT)Temps de Cephalin Kaolin ( TCK)Temps de Cephalin Kaolin ( TCK)

FXII,

FXI

FIX, FVIII

Ca+2

FX,FV,Ca+ 2

II IIa

I Ia

Intrinsic pathway

Kaolin,celite,ellagic acid

PhospholipidPhospholipid

and Caand Ca+ 2+ 2Common pathwayCommon pathway

cephalin

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

Thrombin timeThrombin time

X, V, Pf3

II IIa

I Ia

Final stageFinal stage

Thrombin

ngai sinh

* 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) - Tcau : SL & CL : BT

- PT : BT - aPTT ( > chng 10)

- aPTT hon hp : ngan lai bnh thng

Pbn + Pbt Phh Xn aPTT aPTT hh

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

tien lng benh

Benh nhan Hon hp Benh ly

Thieu hut cac YTM

Khang ong lu

hanh

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

- Nang : 1% - TB : 2-5% -

- Nhe : 5-30% - 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]

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(K2-Farnoquinon), 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, XHN-

MN, 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 : Plasminogen-

Anti 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)


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


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


cac yeu to ong mau

ieu tr :

+ Nguon goc gay suy gan ( neu co the c)

+ Truyen HT ti L khi co chay mau : 20-

30ml/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

KLH trong benh RLM di truyen:

o 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

o Thng benh nhan co TC nang ne hn, khong ap ng vi

T cu. XN hon hp khong rut ngan c

o T le t vong cao, t ap ng corticoit va thuoc C mien

dch

o 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.

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 9 18

Sau phc mc/ ngc 9 5

Sau m 1 11

Khp 7 2

Xut huyt no 3 0

Khc 9 23

Khng xut huyt 4 Khng p dng

T vong 9 Khng d liu

Cc triu chng chy mu bnh nhn

Hemophilia mc phi

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

Yeu to ung cham

XII

XI

IX

VIII

TON THNG MACH MAU HUY HOAI TO CHC

Thromboplastin tissue

VII Pf

3 + Ca++

X

v

II Thrombin

I Fibrin S Fibrin I

XIII

Ca++

S TNG TAC CUA 2 NG ONG MAU

XII

XI

IX

X V

II

THROMBIN

VII

VIIa VIII

V

Va

Thrombin

VIIa

VIII

VIIIa

S KHUYEC AI CUA HIEN TNG ONG MAU

I-II-V-VII

VIII-IX-X

XI-XII

Tieu cau

I

II

V

VIII

Tieu cau

VII

IX

X

XI

XII

MAU TOAN PHAN HUYET THANH CUC MAU ONG

CAC YEU TO ONG MAU B TIEU THU

GIAI OAN TIEU SI HUYET

Plasminogen

Plasmin

fibrin PDF

( X,Y,D,E )

XIIa Yeu to to chc

Anti-plasmin

Vong noi mo

Gan, Lach

Fibrinogen 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

0 im 1 im, 2 im 3 im

S lng tiu

cu

(x 109/L )

100

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 v 120

3

1

0

Thi gian Prothrombin (PT)

- > 1,2

- < 1,2

1

0

Fibrinogen / FDP (mg/ L)

- > 25

- > 10 v

NGUYEN NHAN DIC

Noi khoa : Nhiem trung huyet

Sot ret ac tnh

Benh ly noi khoa giai oan cuoi

Bach cau cap ( M3, va SL > 50.000/mm3)..

Ngoai khoa : a chan thng

Bong o III, > 30% dien tch c the

San khoa : Thai chet lu * Nhiem oc thai nghen

Nhau tien ao * Nhau bong non.

Phoi hp

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

Documents

Benh Dmht ( Bai Giang)