curs 21 coagul
TRANSCRIPT
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SINDROAMELEHEMORAGIPARE
(HEMORAGICE)
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tulburari ale hemostazei (ansamblul fenomenelor fiziologice, careconcura la prevenirea si oprirea hemoragiilor)
hemostaza cuprinde 3 etape:
1. hemostaza primara: timp vascular
timp plachetarleziuni traumatice, chimice sau infectioase ale endoteliului sisubendoteliului vascular vasoconstrictie + activarea trombocitelorsi a factorilor de coagulare (von Willebrand si fibrinogen) fixeazatrombocitele de endoteliu (aderare); activarea trombocitelor consta inmodificari morfologice (aparitia de pseudopode), eliberare de
serotonina, ADP, Ca, factor de crestere derivat din plachete PDGFs.a. din granulele sale si secretia de PG (tromboxan A2 cu puternicefect agregant si vasoconstrictor) si de factor plachetar 3 (FP3) cu rolde activare a coagularii; activarea plachetara este urmata deagregarea acestora, indusa prin fibrinogen, ADP, serotonina,trombina, adrenalina, TxA2 s.a.) cu formarea unui dop hemostatic;acesta apoi este consolidat (retractia cheagului)
2. coagularea plasmatica transformarea plasmei lichide in gel printransformarea fibrinogenului solubil in fibrina insolubila sub actiuneatrombinei; trombina la randul ei este activata din protrombina printr-unlant de reactii enzimatice (cascada coagularii);
3. fibrinoliza indepartarea cheagului de fibrina printr-un mecanismcomplex intre activatori si inhibitori, un rol esential avandu-l plasmina
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anomalii ale hemostazei primare:
- purpure vasculare- anomalii ale trombocitelor
cantitative
calitative- b. von Willebrand
anomalii ale coagularii
anomalii ale fibrinolizei
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Purpurele vasculare (PV)
grup heterogen de maladii, care secaracterizeaza prin sindr. hemoragipar
printr-o afectare a peretelui vascular (prin
defect anatomic congenital, mecanism
imunologic, infectios, necunoscut)
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1.Teleangiectazia familialahemoragica (b. Rendu-Osler)
PV determinata genetic, cu transmitereautosomal dominanta
Morfopatologie
Peretele capilar si cel al venulelor esteredus la un singur strat de celule
endoteliale, incat aceste vase se dilata sise pot rupe
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Tablou clinic
- teleangiectazii leziuni de 1-3 mm, rosii,
care cresc progresiv in dimensiuni; localizatela nivelul pometilor, buzelor, limbii, evt. si pemucoasa digestiva, respiratorie, in ficat sicreier
- manifestari hemoragice epistaxis,hemoptizii, hemoragii digestive
- la nivelul ficatului hemangioame
- la nivelul plamanului fistule arterio-
venoase hipoxie poliglobulie sec. sidegete hippocratice
- evt. + rinichi polichistici, anevrisme cerebrale
http://www.geocities.com/piracawuare/hemangioma.jpghttp://www.orl-france.org/enseignement/Les%20cours/Question313/images313/Rendu%20Osler.jpg -
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Diagnostic
- clinic: teleangiectazii aparute in context
familial + hemoragii
- anemie feripriva
Diagnostic diferential
- stelute vasculare (semnul apasarii)
- petele din polipoza fam. Peutz-Jeghers
(brune)
- sindr. CREST (+ calcinoza, sindr. Raynaud,
disfunctie esof., sclerodactilie)
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Tratament
- trofice capilare- oprirea hemoragiei: electrocoagulare,
fotocoagulare cu laser, hemostaza
locala prin compresiune, hemostaticelocale, excizia leziunii sangerande
- tratamentul anemiei (prep. Fe)
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2. PV imunologice
PV asociate cu anomalii ale Ig
Ig poli- sau monoclonale anormale,
care apar in cadrul altor afectiuni(colagenoze, macroglobulinemie
Waldenstrm, crioglobulinemii)
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Tablou clinic
- tabloul clinic al bolii de baza- purpura petesiala, rar echimotica;
mai ales la nivelul gambelor, evt. si
coapselor si abdom.; se poate agrava la
efort sau frig; evt. necroze cutanate
- evt. + artralgii, mialgii, parestezii,
edeme, fenomen Raynaud; rar afectare
renala (glomerulopatie sau tubulopatie)
sau neurologica (neuropatie periferica)
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Diagnostic
- clinic- hiperglobulinemie, de obicei
policlonala (IgG, IgA, IgM)
- crioglobulinemie- evt. factor reumatoid prezent
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Evolutie
in functie de boala de baza; PV in
principiu evol. buna
Tratament- corticoterapie
- tratam. bolii de baza
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Purpura reumatoida (Schnlein-Henoch)
depuneri de IgA la nivelul leziunilorcutanate posibil rol sensibilizarea la
Agii streptococici sau la medicamente
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Evolutie
benigna, chiar cu vindecari spontane;prognostic rezervat in afectare renala
Tratament- corticoterapie
- evt. Penicilina (ASLO )
- intreruperea tratam. daca se presupune
o alergie medicam.
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3. PV infectioase
in context febril, cu stare generala alterata- Purpura fulminanta
in cadrul unei meningite sau a unei septicemii cumeningococ; la copil; debut brutal, cu agravare rapida;purpura de obicei de tip echimotic, necrotic, diseminata,evt. hemoragii mucoase sau viscerale; evt. sindr.meningian, convulsii, torpoare, chiar coma; frecvent + CID,
cu leziuni necrotice ale extremitatilor; tratam. cuantibiotice, corticoterapie, antipiretice, heparina si plasmaproaspata in CID
- Septicemia stafilococica
purpura centrata de o pustula
- Bolile infectioase eruptive (varicela, rujeola, scarlatina)purpura diferita de exantemul caracteristic bolii, evt. +CID
- Endocardita infectioasa
purpura cu localizare conjunctivala, supraclaviculara;febra + suflu cardiac
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4. PV de cauze diverse
PV din cadrul amiloidozei
Purpura senila (atrofia tesuturilor cutanate sisubcutanate)
Purpura in cadrul unei corticoterapii prelungite
PV din scorbut
Purpura asociata unei staze venoase
mai ales la nivelul gambelor; pete brune, cuelemente purpurice si zone depigmentate
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Trombocitopeniile(Trombopeniile)
scaderea trombocitelor sub 150000/l
Etiopatogeneza
- productie insuficienta prin sindr. de insuf. medulara
(anemie aplastica, leucemii, tratament mielosupresiv,diuretice, alcool, estrogeni, invadare medulara a unortumori solide) sau sindr. dismielopoietic (anemiemegaloblastica, sindr. mielodisplazice), rar prin aplaziesau displazie megacariocitara pura
trombopenii centrale- hiperdistructie periferica (imunologic, hipersplenism),consum exagerat (CID), distructie mecanica (purpuratrombotica trombocitopenica, sindr. hemolitic uremic)
trombopenii periferice
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Tablou clinic
- petesii, echimoze
- epistaxis, gingivoragii
- menoragii, hemoragii digestive
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trombocitopenie
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Diagnostic
- clinic
- teste de coagulare normale, cu exceptiat. Howell prelungit in trombopenii severe
- timp de sangerare
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1.Trombocitopenii autoimune (TAI)
trombocitopenii datorita scaderii durateide viata a trombocitelor prin autoanticorpi
anti-trombocitari
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Etiopatogeneza- se formeaza clone de limfocite B producatoare
de autoanticorpi IgG sau IgM; acesti Aci se
fixeaza pe trombocite; astfel trombocitul esterecunoscut ca nonself si distrus de macrofag
- pe langa trombopenia astfel creata, Aciidetermina si o trombopatie (trombastenie de tip
Glanzmann), care consta intr-un raspunsdeprimat la stimulii agreganti (ADP, colagen,adrenalina)
- uneori Acii actioneaza si asupra megacariocitelordin maduva osoasa, determinand si o
componenta centrala a trombopeniei- in cazul TAI post-medicamentoase si post-
transfuzionale are loc fenomenul de spectatorinocent prin CI depozitate pe trombocite
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Tablou clinic
-petesii, echimoze, sufuziuni
- epistaxis, gingivoragii, metroragii,hemoragii conjunctivale, mai rar hemoragiidigestive, retiniene, cerebrale, renale
- simptomatologia bolii de baza in TAI sec.;trombocitopenia poate fi chiar semn dedebut in colagenoze, SIDA
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Diagnostic- trombocitopenie izolata, de obicei sub 80000/l;
anizotrombocitoza cu predominanta
trombocitelor mari- medulograma numar normal sau chiar
crescut de megacariocite
- evidentierea Aci. anti-trombocitari:
a. direct test anti-globulina cu Aci marcatifluorescenti (IF) sau radioactiv
b. indirect prin incubarea de trombocitenormale cu serul de studiat, urmat de testare
directa reactii fals+- AAN, Aci. anti-ADN, AAM, Aci. anti-HIV,imunofenotiparea limfocitelor pentrudecelarea dif. forme clinice
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Diagnostic diferential
- In medulograma anormala (hipoplaziemegacariocitara): anemie aplastica, sindr.mielodisplazice, leucemii ac. sau cr.,aplazie toxica sau post-chimioterapie
- In medulograma normala: hipersplenism,CID
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Forme clinice
1. TAI idiopatica (Purpura trombocitopenica
idiopaticaPTI)2. TAI secundara asociata altor boli
a. autoimune (LES, PR, tiroidita)
b. maligne (LNH, LLC, tumori solide)
c. SIDA
d. posttransplant de maduva osoasa
e. post-transfuzie
f. post-medicamentoasa3. TAI ac. postvirala la copii, evol. ac. spontan
reversibila
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Tratament in functie de forma clinica in PTI:
- doar tratament simptomatic in trombopenii moderate sifara hemoragii la nivelul mucoaselor
- corticoterapie Prednison 1-1,5 mg/kgc/zi; dupanormalizarea trombocitelor se scade doza progresiv
- splenectomie daca corticoterapia ineficienta sau
contraindicata- daca si splenectomia ineficienta sau contraindicata:
imunosupresoare (blocheaza macrofagele) Vincristin,Vinblastin, Azatioprina, Ciclofosfamida, Danazol; Ig iv. indoze mari (blocheaza receptorii de pe macrofage)
- in urgente( hemoragii severe): 100 mg Prednison, apoicate 25 mg la 6 ore + transfuzii de trombocite cate 6unitati la 6 ore + evt. Vincristin 2 mg; daca ineficient splenectomie de urgenta sau Ig iv. 4-5 zile + transfuziitrombocitare; evt. plasmafereza
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in TAI sec.:
tratam. bolii de baza in TAI ac. postvirala:
daca hemoragii la nivelul mucoaselor
tratam. de urgenta (cortizon, Ig iv.); dacanu doar dispensarizare
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2. Purpura trombotica trombocitopenica(PTT, sindr. Moskovitz)
trombocitopenie prin agregare diseminata,reversibila a trombocitelor
Etiopatogeneza
- defect genetic, transmis autosomal recesiv deficit de depolimeraze (enz. proteolitice) prezenta unor multimeri de factor v. Willebrand
- deficit de activator tisular al plasminogenului la
nivelul endoteliului- prin ambele mecanisme formare de agregate
plachetare diseminate, cu obstruarea vaselormici hipoxie consecutiva
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Tablou clinic
- paloare, icter
- purpura petesiala, hemoragii pe mucoase
- fenomene cerebrale tranzitorii (confuzie,halucinatii, delir, pareze, paralizii, dislalie,
afazie)
- evt. greata, varsaturi, dureri abdom.,hepato-splenomegalie
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Diagnostic
- anemie hemolitica microangiopatica
- trombopenie prin consum
- evt. leucocitoza
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Evolutie si prognostic
netratat evol. severa: 80-90% mortalitateprin hemoragii cerebrale, renale
tratat se vindeca
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Tratament
- plasmafereza + administrare de plasma
proaspata
- evt. corticoterapie, antiagregante
plachetare, Vincristin, Vinblastin
- in IRA HD
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3. Sindr. hemolitic uremic Gasser
mai ales la copii < 6 ani
etiopatogeneza asemanatoare cu cea din
PTT; depozitele de trombocite si fibrina pe
glomeruli determina complicatiile renale
tablou clinic: anemie hemolitica
microangiopatica + proteinurie masiva,
hemoglobinurie si IRA
tratament ca in PTT
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Trombocitopatiile(Trombopatiile)
anomalii rare, care constau in alteraristructurale trombocitare cu tulburari
functionale consecutive in aderarea,
agregarea si secretia trombocitelor
ereditare sau castigate
Trombopatii ereditare
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Trombopatii ereditare
- B. Bernard Soulier: transmisa autosomal recesiv defect demembrana (lipsa complexului GP I) deficit de aderare;clinic hemoragii cutaneo-mucoase din primele luni de
viata; evt. asociata o trombopenie; nu se corecteaza cuplasma normala
- Trombastenia Glanzmann: transmisa tot autosomal recesiv tot defect de membrana (lipsa complexului GP IIb/IIIa) lipsa fixarii fibrinogenului, a retractiei cheagului, deficit in
agregarea la ADP, colagen, trombina; clinic purpuracutaneo-mucoasa din primele luni de viata- B. rezervorului de stocare (storage pool disease)
a) B. granulelor: deficit al granulelor, care stocheazafactorul de crestere derivat din trombocite (PDGF)
eliberare continua de PDGF fibroza medulara cu formarede trombocite mari, de culoare grib) B. granulelor dense (): deficit de stocare al ADP, ATP,serotoninei deficit in agregarea la colagen
- Anomalia de tip aspirinic: deficit de enzime implicate in
sinteza PG diminuarea agregarii la colagen si acidarahidonic
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Trombopatii castigate
sindr. mieloproliferative, sindr.mielodisplazice, purpure trombocitopenice,
anemie Biermer, cardiopatii congen.,
tratam. cu AINS
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Tratament
- contraindicate antiagregante plachetare- in urgenta: transfuzii de masa
trombocitara
- in b. rezervorului de stocare: crioprecipitatde globulina antihemofilica sau 1-dezamino-8-D-arginin-vasopresina
(DDAVP)
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Boala von Willebrand
afectiune congenitala definitaprintr-o anomalie cantitativa sau
calitativa a factorului VIII (von
Willebrand)
Etiopatogeneza
transmitere autosomal dominanta deficit de sinteza sau de polimerizare
a factorului VIII deficit de aderareintre trombocite (complexul GP I) si
straturile endoteliale
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Tablou clinic
hemoragii retro-amigdaliene,
epistaxis, metroragii severe,
echimoze, hematoame,
rar hemoragii digestive
- Diagnostic- TS
- deficit de agregare la ristocetina, carese corecteaza cu plasma normala
- adezivitate plachetara
- Ag. legat de factorul VIII Endothelial cells with stored von
Willebrand factor (red) in the
cytoplasmic granules (Weibal-Palade bodies).
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Tratament
- In formele usoare: DDAVP + evt.antifibrinolitic (EACA) in caz de hemoragii;in hemoragii din cavitati inchise EACA e
contraindicat (impiedica indepartareacheagurilor)
- In formele severe: crioprecipitat de
globulina antihemofilica sau concentrat defactor VIII
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ANOMALIILE COAGULARII
Deficite congenitale in factori de coagulare
Coagularea intravasculara diseminata(CID)
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1. Deficitele congenitale in factori decoagulare
de obicei cu transmitere autosomal recesiva;intereseaza de obicei un singur factor; rare
hemoragii din prima copilarie, proportionalecu gradul defectului
clasificare:1. Deficitele in fibrinformare
2. Deficitele factorilor dependenti de vitamina K
3. Deficitele cofactorilor
4. Deficitele factorilor de contact5. Deficitele inhibitorilor coagularii
1 D fi i l i fib i f
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1. Deficitele in fibrinformare
a) Deficitul in fibrinogen
- afibrinogenemia concentratie
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2. Deficitele factorilor dependenti de vitamina K
a) Deficitul in protrombina
foarte rar
clinic: hemoragii cutaneo-mucoase, la nivelul plagilor, hemartroze
paraclinic: timp de tromboplastina (TTP), TQtratament: complex protrombinic partial activat (CPPA)b) Deficitul in proconvertina (VII)
clinic: hemoragii grave cutaneo-mucoase, cerebrale, hemartroze
paraclinic: TQtratament: CPPA
c) Deficitul in factor X
foarte rar
clinic: hemoragii cutaneo-mucoase si postoperatorii
paraclinic: TTP, TQ
tratament: CPPA
d) Deficitul in factor hemofilic B (IX)e) Deficitele proteinelor C si S
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3. Deficitele cofactorilor (V si VIII)
a) Deficitul de proaccelerina (V)
rar
clinic: hemoragii postoperatorii
paraclinic: TTP , TQ
tratament: plasma proaspata congelata
b) Deficitul de factor VIII
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4. Deficitele factorilor de contact
a) Deficitul factorului Hageman (XII)
nu duce la hemoragii, dar poate favorizatrombozele
b) Deficitele in prekalikreina si kininogen cu
greutate mare (factor Fitzgerald)nu duce la hemoragii
c) Deficitul in factor Rosenthal (XI)
poate cauza hemoragii moderate postoperator,
post-extractii dentare
5 D fi it l i hibit il l ii
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5. Deficitele inhibitorilor coagularii
a) Deficitul antitrombinei (III)clinic: tromboze, tromboflebite, ulcere
varicoaseparaclinic: teste de coagulare normale,
concentratia antitrombinei (AT) 30- 50% dinnormal
tratament: concentrate de ATb) Deficitele de proteina C (PC) si S (PS)
PC si PS proteaze dependente devitam. K
clinic: tromboze
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Hemofiliile
anomalii ale coagularii, caracterizate prindeficit congenital cantitativ sau calitativ de factor
VIII (hemofilia A 80-85%) sau de factor IX
(hemofilia B 15-20%)
Etiopatogeneza
2 gene diferite, ambele pe cromozomul X;
transmitere recesiva legata de sex: purtatoare, prezinta boala clinic
Tablou clinic
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Tablou clinic
In functie de concentratia reziduala a factorului VIII sau IX forme inaparente (25-50%), usoare (5-25%), medii(1-5%) sau severe (
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Diagnostic
- clinic
- ancheta familiara
- TH, TTP
- TQ, TT normale
- test de generare a tromboplastinei (TGT)
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Tratament- local: compresiune prelungita, evt. adaos de
trombina
- tratament substitutiv cu plasma si preparate din
plasma, respectiv concentrate (crioprecipitat deglobulina antihemofilica)
- tratamentul complicatiilor: sinovectomie,kinetoterapie
- tratament profilactic: evitarea traumatismelor,DDAVP preoperator, proteza hemostatica instomatologie
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-
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Tablou clinic
coexistenta manifestarilor hemoragice sitrombotice
- hemoragii cutaneo-mucoase (prin trombopeniesi fibrinogenopenie de consum)
- tromboze cu tulburari neurologice (tulb. deconstienta, coma), cutanate (necroze,gangrene), IRA, detresa respiratorie ac. aadultului
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Diagnostic
- clinic
- TQ
- trombopenie
- fibrinogenopenie
- TTP (toate prin consum exagerat)
- monomeri de fibrina , produsi de degradare aifibrinogenului/fibrinei (PDF) (prin activareasecundara a fibrinolizei)
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Diagnostic diferential
- insuficienta hepatica (fara trombopenie,PDF normali); CID si IH se pot intrica
- fibrinoliza primara din IH, dupa interventiichirurg. pe plaman, uter, prostata
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Tratament
- tratamentul afectiunii cauzatoare de CID
- tratament substitutiv: sange proaspat, plasma
proaspata, concentrate de trombocite, fibrinogenpurificat
- tratament cu heparina: pentru prevenirea de noi
trombi; risc de agravare a sindr. hemoragiparexistent; doze mici 100-300 UI/kgc/zi
- contraindicate: CPPA, antifibrinolitice (EACA)
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ANOMALIILEFIBRINOLIZEI
cu caracter familial
castigate
1 A liil t f ili l l
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1. Anomaliile cu caracter familial ale
fibrinolizei
cu tendinta la hemoragii- Deficitul familial de 2-antiplasmina (b.
Miyasato) foarte rar; transmis autosomalrecesiv; hemoragii din cordonul ombilical,
epistaxis, hematurie, hemartroze, hematoameintramusculare, mai ales hemoragii tardive siprelungite dupa plagi si interventii chirurg.;tratament cu agenti antifibrinolitici (acid -amino-caproic EACA)
- Deficitul de inhibitor al activarii plasminogenului tablou clinic si tratament ca in b. Miyasato
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cu tendinta la tromboza
- Deficitul de activator tisular al
plasminogenului (t-PA)
- Anomaliile familiale ale plasminogenului
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2. Anomaliile castigate ale fibrinolizei
cu fibrinoliza accelerata- Ciroza hepatica si insuficienta hepatica ac.
sindr. hemoragipar prin deficit de sinteza hepatica defactori de coagulare + fibrinoliza accelerata (sintezahepatica deficitara de inhibitor al activarii
plasminogenului si 2-antiplasmina)- LAM
sindr. hemoragipar sever prin CID (eliberarea dinpromielocite a unor enzime capabile sa activeze factoriide coagulare), urmata de fibrinoliza secundara
- toate afectiunile cu CID
- SN hipoalbuminemie presiune coloidosmotica hiperactivitate endoteliala sinteza t-PA
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cu scaderea fibrinolizei
- Reactia de faza acuta staripostoperatorii, afectiuni inflamatorii
hipofibrinoliza prin t-PA si PAI
- Sindr. metabolic, obezitate, DZ,
hiperlipoproteinemii hipofibrinoliza prin
PAI predispune la ateroscleroza