SINDROMUL HEMORAGIPAR

CLASIFICAREA SINDROMULUI HEMORAGIPAR (SH)

I. Vasopatii

II. Trombocitare:- trombocitopenii
- trombopatii
III. Coagulopatii
SH Tabloul Clinic
1. Hemoragii pe mucoase
2. Cutanate
3. Subcutanate
4. Musculare
5. Articulare
6. Viscerale
7. SNC

-Izolate sau asociate.
SH Tabloul Clinic
Alte simptome:

- Febr
- Paloare
- Tahicardie
- Adenopatii
- Hepatomegalie
- Splenomegalie
- Spleno - hepatomegalie

SH Diagnostic diferenial

- Urticaria hemoragic
- Alergii cutanate
- Boli infecto-contagioase cu exantem
- Artrite
- Etc.
DIATEZ HEMORAGIC


Normal Timp de coagulare Prelungit

COAGULOPATII
BOALA VON WILLEBRAND
Normal Timp de sngerare Prelungit


PURPUR VASCULAR
PURPUR SCHONLEIN HENOCH
Numr trombocite


Normal Sczut


TROMBOCITOPENII
ANOMALII CALITATIVE TROMBOCITARE
(genetice sau dobndite)

Sindromul hemoragipar. Explorare
I. Timpul vasculo - plachetar (VN)
TS = 2 4 min.
Trombocite = peste 150.000/mm3
Test garou (Rumpel Leed) sub 10 peteii

II. Coagularea propriuzis
TC = 8 - 10 min
TH = 1 min 20sec. - 2 min. 40 sec.
TQ = 13 14 sec (100%)
APTT = 35 45 sec.


Sindromul hemoragipar. Explorare



Vasopatii
singura anomalie TS uor prelungit
doar la 1/3 din cazuri.

Sindromul hemoragipar. Explorare


SH trombocitare :
- trombocitopenii - tr sub 150.000/mm3
- TS -

- trombopatii: - nr. Tr= N
- TS -
- teste func. Tr. modificate



Sindromul hemoragipar. Explorare
Coagulopatii: TS - N
Tr N

TH N + TQ - deficit calea extrinsec

TH + TQ-N + APTT - deficit
calea intrinsec

Sindroame hemoragice trombocitare
Clasificare
Trombopatii - genetice
- dobndite
Trombocitopenii :
1.Distrucie - imun: - PTI
-S.Evans
-medicamente
-LES
- neimun :CID, inf. virale,etc
2.Distribuie: hipersplenism
3. Scderea produciei
Purpura trombocitopenic imun (PTI)
Etiopatogenez:

CIC (virus + ac. antivirali) alipite de TR macrofag din
splin fagocitare;

Ac. anti-Tr - Tr macrofag splenic fagocitare.
Purpura trombocitopenic imun (PTI)
Tabloul clinic

- Starea general bun , afebril
- Infecie viral cu 1-4 sptmni anterior;
- Peteii
- Echimoze
- Epistaxis
- Gingivoragii, bule hemoragice
- Anemie
- Absena adeno hepato splenomegaliei !!!
Purpura trombocitopenic imun (PTI)
Examinri de laborator

TS prelungit ( 6 60 min.)

Trombocite : < 20.00/mm3 F. Sever
20.00 60.000/mm3 - F. Medie
60.00 100.000/mm3 - F.Uoar

Puncie medular :megacariocite netrombocitogene
Dg. diferenial LAL.
Purpura trombocitopenic imun (PTI)
Evoluie


Acut pn la 6 luni
Recidivant
Cronic - peste 6 luni.
Purpura trombocitopenic imun (PTI)
Tratament
Sub 20.000/mm3 Tr (40.000)
F. severe : IgG 400mg./Kg /zi 5 zile

Methilprednisolon 30 mg/kg/zi- 3 zile.

F. medii/uoare:
Prednison: 1 2 mg/Kg/zi 3 subdoze
10 14 zile
apoi reducerea dozei- sistare.
Transfuzie: MTr, ME

Purpura trombocitopenic imun (PTI)
Tratament

Spenectomia: - F. Cronic
- vaccin anti - pneumococ
- hemophilus
- meningococ

Pentru intervenii chirurgicale- minim 50.000 Tr/mm3

Contraindicaii: aspirina, injecii im, vaccinri 1 an.


Purpura trombocitopenic imun (PTI)



Prognostic: vindecare - 3 luni 75%
- 9 12 luni 90%
cronicizare 10 %.
Boala hemoragic a nou-nscutului
Etiologie :Vit. K ( rezervor, flora din colon)
VIT. K sinteza fact.: II, VII, IX, X, Prot. S, C.

Clinic: Hemoragii
- Melen: unic sau multipl
- Hematoame sc
- Hemoragii din bontul ombilical
- H. vaginal
- Hematurie
- Hemoragie cerebral



Boala hemoragic a nou-nscutului
Forme clinice :

- precoce
- clasic: 2- 3 zi
- tardiv: 1 -3 luni.

Boala hemoragic a nou-nscutului
Tratament
1. Profilactic: vit. K 1mg- im, iv;


2. Curativ: K1, K3 1 2 mg, maxim 5 mg.
im, iv
Plasm : 1o ml/kg la 12 ore.
Coagulopatii Hemofilia A
Genetic:-
- boal gonozomal recesiv
- F VIII C - Xq 28
- femeile purttoare
- brbaii - bolnavi

Coagulopatii Hemofilia A
Clinic:
- Hemartroz
- Hematoame cutanate
- Epistaxis
- Gingivoragii
- Hematurie
- Musculare : psoas
- Zone periculoase: orbitar, faringe, etc.
- SNC

Coagulopatii Hemofilia A
F VIII C valoare normal = 50 150%

Forme clinice :

Sever: F VIII C sub 1 %
Medie: 1 5 %
Uoar: 5 25 %
Coagulopatii Hemofilia A
Examinri de laborator:
TC prelungit
TH prelungit
TQ normal
APTT prelungit
F VIII C - sczut

Coagulopatii Hemofilia A
Tratament
1 ml plasm are 1u FVIIIC i crete nivelul seric cu 2%
perioada de njumtire a F VIII C 8 12 ore.

Hemoragii mici:
10 - 20 u / kg/ doz
(epistaxis, extracie dentar)
2 doze n 24 ore.
Hemoragii mari :
20 50 -75 u/ kg /doz
2 3 doze pe zi
ex: hemartroz 1 3 zile, 25u/kg / doz

Coagulopatii Hemofilia A
Tratament
Preparate :
- plasm ( proaspt, congelat)
- crioprecipitat
- concentrat de F VIIIC: 20 50u/mi
250 -1000u/50 ml un flacon
- F VIII C recombinat: Recombinate, Kogenate
- DDAVP (Desmopresin): 0,3 g /kg / doz
Coagulopatii Hemofilia A

Complicaii:
- HIV, VHB, VHC,
- Ac. F V III C
- artropatia hemofilic
- neurologice, etc.
Coagulopatii Hemofilia A
Tratament adjuvant
Hemartroz : - imobilizare atel
- prednison 1 2mg/ kg /zi, 3 zile
- fizioterapie

Epistaxis: - gelaspon
- EAC 4 x 50 mg /kg / zi po

Gingivoragii: EAC 7 10 zile

Febr : paracetamol CI- aspirin.
Coagulopatii Hemofilia A
Tratament adjuvant

Gesturi interzise :
- intervenii chirurgicale fr pregtire
prealabil (administrare de F VIII C)
cauterizri, extracii dentare, etc

- injecii im, puncie lombar ( fr F VIII )
Profilaxia hemoragiilor
Medic alert
Coagulopatii Hemofilia B

1 ml plasm - 1u F IX crete nivelul seric cu 1 %

Perioada de injumtire - 18 30 ore.

Tratament la 12 24 ore.


Coagulopatii. Boala Willebrand
Genetic : - AD, AR
- cr. 12p
- 1 -2 % din populaie

Clinic : - hemoragii pe mucoase
- epistaxis
- gingivoragii
- metroragii
Coagulopatii. Boala Willebrand
Laborator:
TS prelungit
TC prelungit
Agregare la ristocetin sczut
F VIII : Ag
C
Will
Coagulopatii. Boala Willebrand
Tratament:
Plasm: 10 - 15 ml /kg n hemoragii uoare
20 - 40 ml /kg - n hemoragii severe

Concentrate de F.WILL - Haemate P
Imunate
DDAVP
EAC
Anticoncepionale
Trat. Anemiei
Sfat genetic.
Vasopatiile
Clasificare

I.Genetice: teleangiectazia Rendu Osler _ Weber
S. Louis Bar

II. Dobndite:- permeabilitate
- fragilitate(infecioas, toxic, mecanic)
- vasculite (inflamaie + necroz)
Vasopatiile
Vasculitele
Clasificare

I.Primare : V. Mari B. Takayasu
V. Mijlocii- B Kawasaki
V. Mici purpura Schonlein Henoch

II.Secundare: B. esut conjunctiv- LES
Infecii: VHB,etc
Vasopatii
Boala KAWASAKI
Criterii Cronologie
1. Febra 5 zile 1 spt. .
2.Congestie conjunctival
bilateral 1 spt. .
3 Buze cheilit
Cavit. Bucal limb zmeurie
Roea buco faringian 1 spt. .
4. Adenopatie cervical bilat. 1,5 cm 1 spt. .
5. Exantem polimorf trunchi 2 spt. .
6. Extremiti roea palmo-plantar
edem indurat , descuamare 2 spt. .

Vasopatii
Boala KAWASAKI
Diagnostic pozitiv 5 din 6 criterii


Anatomie patologic.
n primele 10 zile se costituie:
- anevrisme coronariene
- obstrucie coronarian
- necroz miocardic
- deces.
Vasopatii
Boala KAWASAKI
Tratament

1. IgG iv 400 mg/kg/zi
4 zile

2. Aspirin. 80 - 100 mg/ kg/zi n primele 14 zile
5 10 mg /kg /zi -2 luni.
Vasopatii
Boala KAWASAKI
Evoluie :
- acut -10 zile
- subacut 11 25 zile
- convalescen :70 zile

Prognostic: bun dac tratamentul se iniiaz
n primele 10 zile.
Purpura Schonlein Henoch (PSH)
Definiie:
- Vasculit de vase mici
- Mecanism imun
- Clinic: purpur cutanat
asociat cu : manifestri articulare
digestive
renale
Purpura Schonlein Henoch (PSH)

Epidemiologie: - 3 7 ani mai frecvent
- sex M
- primvara i toamna
Purpura Schonlein Henoch (PSH)
ETIOPATOGENEZ
B. imun Ag + Ac + CIC

Ag.: -bacterii: staf. aureu hem., sterptococ hem
- virusuri:
- alimente: pete, ou, carne, etc;
- nepturi de insecte, seruri.

Ac.: IgA ( hiperreactivitate IgA) GN Berger
Complement c. altern- C3a, C3b -vasodilataie,
chemotactism PN.
Purpura Schonlein Henoch (PSH)
ETIOPATOGENEZ

Depunere pe vase mici CIC + C lezare C5-C9
hemoragie

Localizare : - cutanat
- digestiv
- renal ( prognostic)
- articular
Purpura Schonlein Henoch (PSH)
Anatomie patologic
Vasculit + necroz fibrinoid: capilare
arteriole
venule
Edem, infitraie cu PN, Eo
IF: tegument PBR IgA(M), C
Intesin perforare
Renal : GN: PEM
SFS
PE -semilune
Purpura Schonlein Henoch (PSH)
Tabloul clinic
Asocierea:
1.Purpur cutanat
2.Manifestri articulare
3.Manifestri digestive
4.Renale
Semne generale: febr, amigdalit.
Purpura Schonlein Henoch (PSH)
Tabloul clinic
1. Purpura:- 100 %
- simetric
- membre
- periarticular
- fese
- evoluie n pusee (1 -6)
- rar - bule hemoragice
- rar edem al scalpuiui

Purpura Schonlein Henoch (PSH)
Tabloul clinic
2.M. articulare 60 80%
- artralgii
- artrit
- articulaii mari
- fugace
- fr sechele
- edem dureros al labei piciorului
Purpura Schonlein Henoch (PSH)
Tabloul clinic
3.M. Digestive: 60 -80%

Forme uoare : greuri, vrsturi
Forme severe:
- medicale: - vrsturi incoercibile
- colici
- hemetemez
- melen
- chirurgicale: invaginaie, perforaie

Limfadenit mezenteric, apendicit acut.
Purpura Schonlein Henoch (PSH)
Tabloul clinic
4. M. Renale 20 - 40 %
- PBR 80 90%
- debut : spt. 2 4
- s. urinar- hematurie (micro., macro.)

-GN monosimptomatic(n focar) hematurie,
proteinurie;
-GN acuta difuza : 2 - 4 sindr.
-GNP SN impur (hematurie, ret. azotat)

ALTE: hepatice, miocardice, neurologice.

Purpura Schonlein Henoch (PSH)
Laborator
1.Hemostaza NORMAL !!!!
1/3 TS uor crescut
T. garou pozitiv
Tr- N sau

2.Etiopatogenie: - exudat faringian
- ASLO, IDt tuberc.,AgHBs, Ac.antivirali
-T. inflamatorii nespecifice
-T imunologice Ig (IgA ), C3-N, etc.
Purpura Schonlein Henoch (PSH)
Laborator

3.Ex. renale:
- Pe sindroame
- SN: lipide, colesterol,Esbach
- PBR . Hematurie macroscopic peste 3 spt.

4.Alte: R. Gregersen, t. hepatice, ORL,etc.
Purpura Schonlein Henoch (PSH)
Diagnostic
Pozitiv :
Purpur cutanat + 1 3 manifestri

Diferenial:
- purpura
- hematuria
- artrita
- manif. digestive abdomen acut
- afec. cutanate, etc.
Purpura Schonlein Henoch (PSH)
Tratament
1.Igieno dietetic:
- repaus
- regim nefrit (I IV)
hipoalergic
hemoragia digestiv

2.Etiologic: Penicilin (strept. hem)
Oxacilin( staf.aureu)

Purpura Schonlein Henoch (PSH)
Tratament
3. Patogenetic
Prednison:
- indicaii. f. dureroase
f. abdominale
- efect: reduce edemul, previne complicaiile digest.
- eficien : 2/3 din cazuri
- NU influeneaz: - recidivele
- durata bolii
- nu previne GN

Purpura Schonlein Henoch (PSH)
Tratament
3. Patogenetic
Prednison(continuare).
- durata: 10 -14 zile
- doza:1 -2 mg/kg/zi
- HHC: 10 15 mg/kg/zi iv
4 prize
3 10 zile

Antiagregant: dipiridamol 1-2mg/kg/zi

Purpura Schonlein Henoch (PSH)
Tratament
4.Simptomatic:
- febra
- capilarotrofic
- SDA, etc

5. GNP

Purpura Schonlein Henoch (PSH)
Evoluie: 1 6 pusee

Prognostic: - imediat- complicaiile digestive
- tardiv GNP (20-40%):
50- 90% vindecare
50% - 10% cronicizare
- deces: hemoragie digestiv
Purpura Schonlein Henoch (PSH)

Externare :
- asanarea focarelor ORL sub protecie de
antibiotice;

- bilan renal la 6 luni i un an.

Sindromul hemoragipar. Explorare
I. Timpul vasculo - plachetar (VN)
TS = 2 4 min.
Trombocite = peste 150.000/mm3
Test garou (Rumpel Leed) sub 10 peteii

II. Coagularea propriuzis
TC = 8 - 10 min
TH = 1 min 20sec. - 2 min. 40 sec.
TQ = 13 14 sec (100%)
APTT = 35 45 sec.


DIATEZ HEMORAGIC


Normal Timp de coagulare Prelungit

COAGULOPATII
BOALA VON WILLEBRAND
Normal Timp de sngerare Prelungit


PURPUR VASCULAR
PURPUR SCHONLEIN HENOCH
Numr trombocite


Normal Sczut


TROMBOCITOPENII
ANOMALII CALITATIVE TROMBOCITARE
(genetice sau dobndite)

Sindromul hemoragipar. Explorare
Coagulopatii: TS - N
Tr N

TH N + TQ - deficit calea extrinsec

TH + TQ-N + APTT - deficit
calea intrinsec