Professional Documents
Culture Documents
I. Etapa vasculara
II. Etapa trombocitara
III. Etapa coagulare
IV. Etapa fibrinoliza
HEMOSTAZA NORMALA
I. Endoteliul
- functia
1. bariera de permeabilitate
2. secretie:
- F. Willebrand,
- trombomodulin ataseaza trombina, proteina C, S
- fibronectina
inhibitorul activatorului tisular al
plasminogenului
- PGI2 , Endotelina
subendotelial
- fibronectin + f. Willebrand
prin
Gp IIb IIIa
- agregarea
- ADP subendotelial
- fibrinogen
- Tx A 2
- secretia
- Ca +2 , Factor 3, 4,
COAGULAREA - intrinseca
X a
- trombocite activate
colagenul subendotelial
factorul XII
- XII
XII a
- XI
XI a
- IX
Ca, F3plachetarfosfolipide,VIII
- X
COAGULAREA - extrinseca
- tromboplastina tisulara
FT
- VII
Xa
- X
Proteina C
Xa
- X
Proteina C
IIa
- II
Ca, F3plachetar,
V
- fibrinogen
fibrina
+
fibrinopeptid
COAGULAREA
CLASIFICAR
E
- Etapa vasculara - Purpura
vasculara
- congenitala
-
dobandita
- Etapa trombocitara - Purpura
trombocitara
trombocitopenie
- trombopatii
dobandita
- Etapa fibrinolizei - Purpura
fibrinolitica
- Etapa reologica - Purpura
ortostatica
- Etapa activare
sistem monocit-macrofag Purpura CID
Purpure vasculare
- congenitale
Ehler Danlos
osteogeneza imperfecta
Rendu - Osler
- dobandite
scorbut
senila
Sindrom Henoch Schonlein
infectioasa - meningococcica, virale
disglobulinemii - Waldenstrom,
mielom, disglobulinemie mixta
fistule arteriovenoase
(mucoasa digestiva, plaman, ficat, splina,
cerebral)
Telangiectazia ereditara
Telangiectazia ereditara
Manifestari clinice
1. telangiectazii cutanate, mucoase
2. epistaxis, hemoptizii, melena
3. anemie feripriva cr
4. anevrisme arteriovenoase hepatice
cu sindrom hiperkinetic
cardiovascular
5. AHC sugestive boala Rendu
Diagnostic diferential
1. Telangiectaziile sclerodermie
(CREST)
2. Pete telangiectatice
3. B Fabry (Angiokeratoma
corporis
difusum)
- boala a metabolismului glicolipidic
(ceramide intramacrofagic)
CREST
Boala
Fabry
Pete telangiectatice
Tratament
1. Suportiv: tamponament nazal,
unguente ( dermatol),
cauterizari contraindicate
2. Trat anemiei : fier, ac folic,
2. Purpure vasculare
anomalii dobandite
ale tesutului
conjunctiv
Purpura de staza
Purpura senila
Scorbutul
Situatii etiologice
- alcoolism, copii hraniti exclusiv
cu lapte de vaca
- patogenie:
- deficit vit C deficit de colagen
(hidroxiprolina)
- reduce adeziunea, agregarea
plachetelor,
- clinic :
- purura in jurul
foliculului pilos, durere
- hipercheratoza
- rar: HDS,
hemoragii musculare
3. Purpure vasculare
dobandite
- boli infectioase
- boli de tesut
conjunctiv
- hiper/disglobulinemii
- Henoch
Schonlein
Purpure vasculare
dobandite boli inflamatorii
- etiopatogenie
- complexe imune
circulante
- post infectii
bacteriene, virale, rickettsi
- medicamente penicilina, sulfamide,
allopurinol
Purpura infectioasa
- infectii (virusuri, meningococ, rickettsi)
- patogenie: complexe imune circulante
- clinic :
- purura bule hemoragice
purpura fulminans
tratament antiinfectios
- patogenie:
- copii, adolescenti
- dupa infectii
(E Coli, Shigella)
- clinic:
- purpura
- insuf renala acuta
- HTA
- AH microangiopatica
- evolutie :
- deces prin IRA
Meningococcemie
Purpura fulminans
post zona zoster
Endocardita bacteriana
Vasculita poliartrita
reumatoida
Purpura hiperglobulinemica
IgA+IgG
- boala
Waldenstrom, mielom
Manifestari clinice
1. purpura
2. hemoragii retiniene
3. nevrita periferica
Tratament
1. Tratatamentul mielom, etc
2. Plasmafereza
Purpura hiperglobulinemica
Hemoragii subunghiale
- crioglobulinemie
(faringiana 50%cazuri,
Manifestari clinice
Manifestari clinice
1. purpura
2. poliartralgii
3. colica abdominala, melena,
intestinala
rar perforatia
4. Hematurie, proteinurie
GNA ac
Prognostic
mesangiala
focala
favorabil in general
Tratament
1. Penicilina infectie streptococica
2. Corticoterapie 1mg/kg
prednison
Prognostic
- favorabil in general
- evolutie cu recidive purpurice
Complicatii
- IRA
- perforatie intestinala
- anticoagulante
- autoflagelare
- staza
Necroza cumarinica
Purpura trombocitopenica
I. Productie scazuta de
trombocite
3. sindr mielodisplazice:
idiopatica
secundara: radiatii, medicamente,
virusuri
4. anomalii congenitale:
pancitopenia Fanconi,
trombocitopenia autosomal recisiva,
boala May Hegglin, infectii (rubeola)
1. Distructie crescuta :
Trombocitopenia autoimuna
idiopatica
- terminologie
- trombocitopenie imuna primara
- trombocitopenie imuna
primara
persistenta 3-12 luni
- trombocitopenie cronica
Trombocitopenia autoimuna
idiopatica
- etiopatogenie
- autoanticorpi in plasma si pe
membrana trombocitelor
- distrugere prematura a
trombocitelor in macrofagele
splenice
Purpure trombocitopenica
autoimuna idiopatica acuta
- etiopatogenie
IgG
- autoanticorpi antitrombocitari
Manifestari clinice
1. purpura
2. poliartralgii
3. colica abdominala, melena,
ntestinala
rar perforatia
4. Hematurie, proteinurie
GNA ac
mesangiala focala
Manifestari clinice
- clinic
- paraclinic
- TS prelungit
- trombocitopenie
Maduva:
nu megcariocite
trombocitogene
- evolutie
- boala autolimitata cu vindecare,
- uneori cu purpura in valuri
- 20% cronicizare
- tratament
Tratament linia II
1.Splenectomia clasica sau
laparoscopic
-corticorezistenta 6 luni-recidivanti
Tratament linia II
2.Contraindicatii refuz splenectomia
-Azatriopina, Ciclofosfamida, Vincristin,
Ciclosporina A
-Danazol
- Rituximab
-in urgente dupa masa trombocitara
-Agonistii receptorilor de
trombopoietina
Romiplostim s.c.
Purpure trombocitopenica
autoimuna idiopatica cronica
- etiopatogenie
- anticorpi antitrombocitari
anti receptori IIb-IIIa,
glicoproteina Ib
Purpure trombocitopenica
autoimuna idiopatica cronica
- clinic
- femei / barbati = 3/1
- astenie, petesii - nepalpabile,
fara
bule hemoragice,
echimoze,
- metroragii,
sangerari digestive,
fara splenomegalie
- paraclinic
- trombocitopenie
- maduva: megacariocite tinere
- paraclinic
- TS prelungit
- trombocitopenie
- maduva: megacariocite
tinere
numeroase oprite in
maturatie,
Evolutie
rare
Tratament
- cortioterapie 1mg/kg/zi
- 50% remisiune partiala,raspuns la 6
- imuran 3 luni +/- vinblastin
corticorezistente
Purpure trombocitopenica
alloimuna
- postnatala
- anticorpi antitrombocitari ce trec
placenta
A1
- clinic
- anti sistemului PI
Purpure trombocitopenica
posttransfuzionala
- anticorpi antitrombocitari
A1
- anti sistemului PI
- clinic
- la 7 zile de la transfuzie, masa
trombocitara
- tratament
- corticoterapie, plasmafereza,
exanguinotransfuzie
Purpure trombocitopenica
postmedicamentoasa
- actiune maduva osoasa
- arabinosid, daunorubicina,
- tiazidice, estrogeni
- distructie imuna
- antibiotice, sulfamide,
- chinidina, chinina
- metildopa
- saruri de aur,
- heparina
Purpure trombocitopenica
postmedicamentoasa
- mecanism
1. complexe imune
anti
medicament + proteina
plasmatica
- absorbtie nespecifica a complexelor
pe trombocit inocent bystander
2. medicament fixat pe trombocit,
anticorpi anti
complex
medicament + trombocit
- clinic
- petesii, frecvent sindrom
hemoragic sever,
- aparut post
medicamentos
- paraclinic
- megacariocite normale,
intens trombogene ( citoplasma
granulata,
- tratament
- intreruperea medicatiei,
corticoterapie,
plasmafereza,
Purpure trombocitopenica
autoimuna secundara
- LES
- LLC, limfoame
- HIV
- paraclinic
- trombocitopenie
- maduva: megcariocite normale,
anomalii morfologice
- tratament
- zidovudin
Purpure trombotica
trombocitopenica
- context
- sarcina, anticonceptionale, infectii
- Coxackie, micoplasme,
- antibiotice
- morfopatologic
- microtromboza hialina capilara,
arteriolara
Purpure trombotica
trombocitopenica
- clinic
- febra
- purpura
- anemie hemolitica
microangiopatica
- disfunctie renala severa,
- manifestari neurologice
- tratament
- CID
Sindroame hemoragipare
prin
anomalii de coagulare
si
fibrinoliza
dobandita
- congenitala
-
COAGULAREA - intrinseca
colagenul subendotelial
factorul XII
XII
kininogen
precalicreina
Complex la nivelul
endoteliuui
XII a
XI
XI a
IX
IX a
COAGULAREA - intrinseca
colagenul subendotelial
factorul XII
XII
XII a
XI
kininogen
precalicreina
XI a
IX
IX a
COAGULAREA - extrinseca
VIII
VIIIa
Ca, F3plachetar
IIa
fibrina
II
fibrinogen
Fibrinoliza
Pro UK
+
tPA
Plasminogen
Plasmina
PAI
Fibrina
trombina
PD Fibrina
Anticoagularea naturala
X
Xa
Antitrombina
IIa
Inactiveaza
Va
Proteina C a
Proteina S
fibrina
II
Inactiveaza VIIIa
fibrinogen
Proteina C
Trombomodulina + trombina
Hemofilia A X
Generatia I
Generatia
II
x
X
x
xX
x
Generatia
III
Generatia
IV
x
X
Xx
x
X
x
- Clinic
- copii - baieti, tineri
- hematoame, hemartroza,
- hemoragii: muschi,
orofaringe,
cerebrale
Hemofilia A
Hemartroze hemofilia A
- Diagnostic paraclinic
- T sangerare
= normal
- T coagulare
= prelungit
- T protrombina = normal
- AND fetal
- Forme
- severe .. 1%
- medii
- usoare
activitate f VIII
Tratament
Profilactic de 2X pe saptamana
Curativ
Kogenate 1000 UI 15 fl
Octanate 500 UI 13 fl
1UI/kgc factor VIII creste concentratia
plasmatica cu 1%
Tratament
Forme medii
- crioprecipitat plasma
Forme usoare
VIII
Tratament
Forme severe
- concentrat de factor VIII
monoclonali
- cu pretestarea inhibitorului F
pentru nivel 50% a FVIII,2 sa
- adm la interval d
- profilactic
- la simptome, fara
hematoame,
a redus
IX
IX
TS - prelungit
agregare plachetara la ristocetina= absenta
APTT normal, T Quik-normal
concentratia de F Willebrand - scade
- BvW tip II a -
- Clinic:
- femei si barbati
- hemoragii cutanate si mucoase
- forme:
- usoare, medii, severe
- DG. paralinic:
- TS - prelungit
- Agregare plachetara la ristocetina= absenta
- APTT normal, T Quik-normal
- Concentratia plasmatica F Willebrand - scade
-
- Tratament
- concentrate de F VIII
- menoragia
contraceptive orale
- DDAVP sau desmopresina (vaspresina)
creste retentia de
fluide stimuleaza
eliberarea FvW
Deficienta factor XI
-
II
-
Afibrinogenemia si disfibrinogenemia
- Clinic
sangerare
- tromboze in
disfibrinogenemie (rar)
- Paraclinic
- afibrinogenemie: scaderea concentratie de fibrinogen
- disfibrinogenemie:
- Timp
protrombina prelungit
- Timp tromboplastina prelungit
- Tratament
- Timp trombina prelungit
1. Deficienta dobandita a
factorilor de coagulare
- vitamina K - plante
- sintetizata de flora intestinala
epoxidaza
vit K epoxid
vit K
reductaza
Anticoagulanti
cumarinici
f
f
f
f
II
VII
IX
X
- proteina C si S
- antagonisti de vit K
deficienta de vit K
- forme usoare - severe
- Cauze de sangerare
in boli de ficat
- scade sinteza : fibrinogen,
Factori etiologici
XII a
coagulare
fibrinoliza
Activare coagulare in
microcirculatie
Tromboze
XII a
Fibrinoliza in exces
Sangerare difuza
Tromboze
Sangerare difuza
- petesii,
- sangerare - pe mucoase (gastrointestinala, hemoptizie
epistaxis)
- neurologica
Coagulare intravasculara
diseminata
Coagulare intravasculara
diseminata
- Tratament
- tratamentul bolii de baza
- reechilibrare hidroelectrolitica
- heparina 1000 UI/ora, 5000 UI la 4ore
- EACA = 4-6g/24 ore
( blocheaza PDF)
schizocite
- Fibrinoliza
activator tisular plasminogen
PAI
plasminogen
tromb
2 anti plasmina
plasmina
fibrina
PDF
- Fibrinoliza primara
- deficitul 2 anti plasmina
- sindrom hemoragipar
- paraclinic
- scade mult fibrinogenul
- aPTT, T Quick aproape normale
- trombocite normale
- tratament
- plasma proaspata, EACA
ELEMENTE CLINICE
DIAGNOSTIC DIFERENTIAL
- Sindromul hemoragipar
- varsta, sex
- conditii de declansare
- purpura
- localizare
- leziuni palpabile, buloase,
micronecrotice
- acuta, cronica
- hemoragii: mucoase, articulare, SNC
ELEMENTE PARACLINICE
DIAGNOSTIC
- TimpDIFERENTIAL
sangerare
- Trombocite
- Timp coagulare
-
fibrinogen
aPTT
t Quick
concentratie f VIII, IX,
von willebrand
- PDF
- liza cheagului