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HEMOSTAZA NORMALA I. Etapa vasculara II. Etapa trombocitara III. Etapa coagulare IV. Etapa fibrinoliza

Sindroame hemoragipare 2016

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Page 1: Sindroame hemoragipare 2016

HEMOSTAZA NORMALA

I. Etapa vasculara

II. Etapa trombocitara

III. Etapa coagulare

IV. Etapa fibrinoliza

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HEMOSTAZA NORMALA

I. Endoteliul- functia

1. bariera de permeabilitate2. secretie:- F. Willebrand,

- trombomodulin - ataseaza trombina, proteina C, S

- fibronectina – inhibitorul activatorului tisular al plasminogenului - PGI2 , Endotelina

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- adeziunea prin receptori Gp I - colagenul subendotelial

- fibronectin + f. Willebrand prin Gp IIb IIIa

- fibrinogen

II. Etapa trombocitara

- agregarea - ADP subendotelial - Tx A2

- secretia - Ca+2 , Factor 3, 4, serotonina

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COAGULAREA - intrinseca - trombocite activate

colagenul subendotelial factorul XII

- XII XII a - XI XI a

- IX IX a

- X Xa

Ca, F3plachetar- fosfolipide,VIII

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COAGULAREA - extrinseca

- tromboplastina tisulara

FT

- X Xa

Proteina C- VII

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- fibrinogen fibrina +

fibrinopeptid A,B

COAGULAREA - calea comuna

- X Xa

- II IIa

Ca, F3plachetar,V

Proteina C

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COAGULAREA

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CLASIFICARE

- Etapa vasculara - Purpura vasculara- congenitala

- dobandita

- Etapa trombocitara - Purpura trombocitara

- trombocitopenie - trombopatii

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- Etapa coagulare - Purpura deficienta factorilor de coagulare

- congenitala - dobandita

- Etapa reologica - Purpura ortostatica

- Etapa fibrinolizei - Purpura fibrinolitica

- Etapa activare sistem monocit-macrofag - Purpura CID

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Purpure vasculare

- congenitaleEhler Danlos osteogeneza imperfectaRendu - Osler

- dobanditescorbutsenilaSindrom Henoch Schonleininfectioasa - meningococcica, viraledisglobulinemii - Waldenstrom, mielom,

disglobulinemie mixta

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Boala Rendu Osler- congenitala

autosomal dominantadefect perete venos

(endoteliu, membrana bazala, adventice)

fistule arteriovenoase(mucoasa digestiva, plaman, ficat, splina,

cerebral)

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Telangiectazia ereditara

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Telangiectazia ereditara

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Manifestari clinice

1. telangiectazii cutanate, mucoase 2. epistaxis, hemoptizii, melena 3. anemie feripriva cr4. anevrisme arteriovenoase hepatice cu sindrom hiperkinetic cardiovascular 5. AHC – sugestive boala Rendu

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Diagnostic diferential

1. Telangiectaziile sclerodermie (CREST)

2. Pete telangiectatice3. B Fabry (Angiokeratoma corporis

difusum) - boala a metabolismului glicolipidic (ceramide intramacrofagic)

- leziuni nodulare inchise la culoare ( aproape negre) periombilical,

scrotal, cardiac, sistem nervos

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CREST

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Boala Fabry

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Pete telangiectatice

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Tratament

1. Suportiv: tamponament nazal, unguente ( dermatol), cauterizari contraindicate

2. Trat anemiei : fier, ac folic, vit B12 la 3 luni

3. Trat chirurgical al fistulelor mari – greu de abordat

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2. Purpure vasculare anomalii dobandite

ale tesutului conjunctiv

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Purpura de staza

Purpura senila

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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 - tratament: Vit C iv

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3. Purpure vasculare dobandite

- boli infectioase - boli de tesut conjunctiv

- hiper/disglobulinemii - Henoch Schonlein -cauze rare

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Purpure vasculare dobandite – boli inflamatorii

- etiopatogenie

- complexe imune circulante - post infectii bacteriene, virale, rickettsi- medicamente – penicilina, sulfamide, allopurinol

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Purpura infectioasa

- infectii (virusuri, meningococ, rickettsi)

- patogenie: complexe imune circulante - clinic :

- purura – bule hemoragice – “purpura fulminans “ - tratament – antiinfectios

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Sindromul hemolitic uremic

- microtromboze exclusiv in vase renale

- etiologie: infectioasa

- patogenie: - copii, adolescenti- dupa infectii (E Coli, Shigella)

- leziune de celule endoteliale, microtromboze a. aferenta, glomerul

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Sindromul hemolitic uremic

- microtromboze exclusiv in vase renale

- etiologie: infectioasa

- clinic: - purpura

- insuf renala acuta - HTA

- AH microangiopatica- evolutie :

- deces prin IRA - 10- 50% cazuri IRC

- tratament - dializa in IRA

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Meningococcemie

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Purpura fulminans post zona zoster

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Endocardita bacteriana

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Vasculita poliartrita reumatoida

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Manifestari clinice1. purpura2. hemoragii retiniene3. nevrita periferica Tratament

1. Tratatamentul mielom, etc

2. Plasmafereza

Purpura hiperglobulinemica IgA+IgG - boala

Waldenstrom, mielom

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Purpura hiperglobulinemica

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Hemoragii subunghiale - crioglobulinemie

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Purpura Henoch Schonlein- etiopatogenie

infectie streptococica (faringiana – 50%cazuri, respiratorie)

-alergii alimentare, medicamentoase – (chinidina, sulfamide)

membrana bazala capilara – - complexe imune IgA2

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Purpura Henoch Schonlein

Manifestari clinice

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Manifestari clinice1. purpura2. poliartralgii3. colica abdominala, melena,

rar perforatia intestinala

4. Hematurie, proteinurie – GNA ac mesangiala focala Prognostic

favorabil in generalevolutie cu “valuri purpurice”

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Tratament1. Penicilina – infectie streptococica

2. Corticoterapie – 1mg/kg prednison

Prognostic

- favorabil in general- evolutie cu “recidive purpurice”

- IRA - perforatie intestinala

Complicatii

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Purpure vasculare rare

- anticoagulante

- autoflagelare

- staza

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Necroza cumarinica

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Purpura trombocitopenicaI. Productie scazuta de trombocite- trombocitopenia < 100000/mm

3

- Nr redus de megacariocite in maduva1. infiltrat medular : leucemii,

limfoame, mielom, metastaze, granuloame

2. hipoplazie medulara: idiopaticasecundara: radiatii, medicamente,

virusuri

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4. anomalii congenitale: pancitopenia Fanconi, trombocitopenia autosomal recisiva, boala May Hegglin, infectii (rubeola)

3. sindr mielodisplazice: idiopaticasecundara: radiatii, medicamente,

virusuri

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- scurtarea duratei de viata a trombocitelor

1. Distructie crescuta : - PTI autoimuna acuta, cronica

- purpura posttransfuzionala

- neonatala alloimuna- post medicamentoasa- autoimuna secundara

- trombocitopenia < 100000/mm

3

- nr crescut de megacariocite in maduva

II. Distructie crescuta trombocite:

Page 52: Sindroame hemoragipare 2016

Trombocitopenia autoimuna idiopatica

- terminologie- trombocitopenie imuna primara

- trombocitopenie imuna primara persistenta 3-12 luni

- trombocitopenie cronica

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Trombocitopenia autoimuna idiopatica

- etiopatogenie- autoanticorpi in plasma si pe membrana trombocitelor

- distrugere prematura a trombocitelor in macrofagele splenice

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Purpure trombocitopenica autoimuna idiopatica acuta

- etiopatogenie

- autoanticorpi antitrombocitari IgG

- antigene : virus gripal, rubeolic, hepatitic, HIV, Ebstein Barr,

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Manifestari clinice

1. purpura2. poliartralgii3. colica abdominala, melena,

rar perforatia intestinala

4. Hematurie, proteinurie – GNA ac mesangiala focala

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Manifestari clinice

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- clinic- petesii fara splenomegalie

- paraclinic

- trombocitopenie- maduva: megcariocite numeroase

oprite in maturatie,

- TS prelungit

- IgG antiitrombocitari

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Maduva:

nu megcariocite trombocitogene

Page 59: Sindroame hemoragipare 2016

- evolutie- boala autolimitata cu vindecare, - uneori cu purpura “in valuri” - 20% cronicizare

- tratament- cortioterapie in cazuri grave

- plasmafereza in risc de hemoragie cerebrala

Page 60: Sindroame hemoragipare 2016

Tratament linia I la T‹ 30 0001.Corticoterapia

- Prednison 1-1,5 mg-kgc-zi - Dexametazona 40 mg 4 zile la 28 zile 1-4X - Metilprednisolon pulsterapie 500-1000mg 3 zile lunar 2.Imunoglobuline iv urgente, pre-splen. - Ig in doza mare 1g-kgc 2 zile sau 400mg 5 zile - Ig anti-D pacienti Rd pozitivi

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Tratament linia II1.Splenectomia clasica sau laparoscopic-corticorezistenta 6 luni-recidivanti-se poate face la › 50 000 (corticoterapie, Ig.,agonisti ai receptorilor de trombopoietina)-in urgente dupa masa trombocitara - Vaccinare anti S. pneumoniae, N. meningitidis, Haemophilus influenzae

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Tratament linia II2.Contraindicatii –refuz splenectomia-Azatriopina, Ciclofosfamida, Vincristin, Ciclosporina A-Danazol- Rituximab-in urgente dupa masa trombocitara -Agonistii receptorilor de trombopoietina Romiplostim s.c. Eltrombopag p.o.

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Purpure trombocitopenica autoimuna idiopatica cronica

- etiopatogenie

- anticorpi antitrombocitari anti receptori IIb-IIIa, glicoproteina

Ib

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- clinic

- astenie, petesii - “nepalpabile”, fara bule hemoragice, echimoze, - metroragii, sangerari digestive, - fara splenomegalie - paraclinic

- trombocitopenie- maduva: megacariocite tinere

Purpure trombocitopenica autoimuna idiopatica cronica

- femei / barbati = 3/1

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- paraclinic

- trombocitopenie

- maduva: megacariocite tinere numeroase oprite in

maturatie,

- TS prelungit

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Evolutie- cronica, remisiuni spontane f rare

Tratament- cortioterapie 1mg/kg/zi- 50% remisiune partiala,raspuns la 6 sap

- splenectomie – forme corticorezistente - agonisti de trombopoietina – forme corticorezistente

- imuran – 3 luni +/- vinblastin

- gama globulina iv – 3luni blocarea sistemului monocit-macrofag

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- postnatala

- clinic- sindrom hemoragipar imediat dupa nastere

- manifestari grave la urmatorii copii

Purpure trombocitopenica alloimuna

- anticorpi antitrombocitari ce trec placenta

- anti sistemului PI

A1

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- clinic

Purpure trombocitopenica posttransfuzionala

- la 7 zile de la transfuzie, masa trombocitara

- anticorpi antitrombocitari - anti sistemului PIA1

- tratament- corticoterapie, plasmafereza,

exanguinotransfuzie

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- actiune maduva osoasa

Purpure trombocitopenica postmedicamentoasa

- arabinosid, daunorubicina,- tiazidice, estrogeni

- distructie imuna- antibiotice, sulfamide,

- chinidina, chinina- metildopa

- saruri de aur, - heparina

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- mecanism

Purpure trombocitopenica postmedicamentoasa

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”

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- clinic- petesii, frecvent sindrom

hemoragic sever, - aparut post medicamentos

- paraclinic

- intreruperea medicatiei, corticoterapie, plasmafereza,

- tratament

- megacariocite normale, intens trombogene ( citoplasma granulata, azurofila, fara vacuole)

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TIH- sindrom protrombotic indus de un anticoagulant

Tip I primele 4 zile

- de cauza imuna, severa, progresiva - tromboze arteriale, venoase

Tip II dupa 5-15 zile

- datorata aglutinarii T, usoara, limitata

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- paraclinic- trombocitopenie- maduva: megcariocite normale, anomalii morfologice

Purpure trombocitopenica autoimuna secundara

- LES - LLC, limfoame

- HIV

- tratament- zidovudin

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Purpure trombotica trombocitopenica

- sarcina, anticonceptionale, infectii - Coxackie, micoplasme, - antibiotice

- morfopatologic

- context

- microtromboza hialina capilara, arteriolara

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Purpure trombotica trombocitopenica

- clinic- febra

- purpura - anemie hemolitica microangiopatica

- disfunctie renala severa, - manifestari neurologice

- tratament

- CID

Page 76: Sindroame hemoragipare 2016

Sindrom hemolitic uremic

- clinic- copii, tineri

- tratament

- IRA, sindr hemoragipar, rar sindrom neurologic

- prognostic- 50-80% mortalitate

- plasmafereza, exanguinotransfuzia

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Sindroame hemoragipare prin

anomalii de coagulare si

fibrinoliza

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- Etapa coagulare - Purpura deficienta factorilor de coagulare

- congenitala - dobandita

-Etapa fibrinolizei - Purpura fibrinolitica

- anomalii primare ale fibrinolizei- anomalii secundare: CID

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COAGULAREA - intrinseca

colagenul subendotelial factorul XII

XII XII akininogenprecalicreina

XI XI a

IX IX a

X Xa

“Complex” la nivelul endoteliuui

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COAGULAREA - intrinseca

XII XII akininogenprecalicreina

XI XI a

IX IX a

X Xa

COAGULAREA - extrinsecaVIIIa

Ca, F3plachetar

(1)factor tisular + F VII + Ca

VIII

colagenul subendotelial factorul XII

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fibrinogen fibrina

Faza comuna a in coagularea intrinseca si extrinseca X Xa

II IIa

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Fibrinoliza

+tPA

Pro UK

PAI

trombina

Plasminogen

Plasmina-

+

+

Fibrina

PD Fibrina

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fibrinogen fibrina

Anticoagularea naturala

X Xa

II IIaInactiveaza Va

Proteina CProteina S

Proteina C a

Trombomodulina + trombina

Inactiveaza VIIIa

Antitrombina

Page 85: Sindroame hemoragipare 2016

Sindrom hemoragic prin anomalii de coagulare

1. Hemofilia A deficienta factor VIII

- anomalia genei F VIII – bratul lung cromozom X - mutatii punctiforme, deletii importante

- boala afecteaza aproape exclusiv barbatii, deoarece la femei heterozigote cromozomul X asigura sinteza necesara de f VIII

- incidenta 1/ 10 000

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Hemofilia A - deficienta factor VIIIX

y xx

Xy

Xyx

xXy

Xy

Xy x

xx

xX Xy

xx

xxX

XX X

y

Generatia I

Generatia II

Generatia III

Generatia IV

X

Xy

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Factorul VIII – proteina 80 000 daltoni, pe suprafata f. von Willebrand

F VIII – sintetizat in ficat- concentratie plasmatica = 10 g/l

- durata de viata 8-12 ore

Hemostaza normala necesita activitate – 25% f VIII

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- Clinic

- copii - baieti, tineri - hematoame, hemartroza,

- hemoragii: muschi,

orofaringe, cerebrale

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Hemofilia A

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Hemartroze – hemofilia A

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- Diagnostic paraclinic

- T sangerare = normal - T coagulare = prelungit - T protrombina = normal - T tromboplastina partial

activata = prelungit

- AND fetal

- Concentratie F VIII = scazuta

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- Forme- severe …….. 1% activitate f VIII

- medii …….. 1-5% activitate f VIII

- usoare …….. 5% activitate f VIII

- f usoare .… 5 - 25% activitate f VIII

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Tratament

• Profilactic de 2X pe saptamana• CurativKogenate 1000 UI 15 flOctanate 500 UI 13 fl• 1UI/kgc factor VIII creste concentratia

plasmatica cu 1%

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Tratament

- crioprecipitat plasma Forme medii

Forme usoare

- desmopresin – creste nivelul F VIII

- EACA – inainte de extractii dentare inhibitor de fibrinoliza ( inhiba activatorul tisular plasminogen)

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Tratament

- concentrat de factor VIII - prin absorbtie pe anticorpi monoclonali sau f VIII recombinat

- chirurgia majora, ortopedica - cu pretestarea inhibitorului FVIII – pentru nivel 50% a FVIII,2 sapt - adm la interval de 8 ore sau 12 ore

- profilactic - la simptome, fara hematoame, a redus hemartroza

Forme severe

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1. Hemofilia B – boala Christmas deficienta factor IX

- Anomalia genei IX – bratul lung cromozom X

- mutatii punctiforme, 1/3 spontane- microdeletii

- 800 pattern- uri de anomalii moleculareex: factor IX Leiden – 11 mutatii punctiforme

- Factor IX - sinteza hepatica dependenta de vit. K

- 20% din hemofilii

Page 98: Sindroame hemoragipare 2016

1. Hemofilia B – boala Christmas deficienta factor IX

- Paraclinic – concentratia fact IX < 1%

- Tratament – plasma proaspata, - complex protrombinic liofilizat ( risc de tromboze)

- Clinic – sindrom hemoragipar expresie fenotipica variabila

- ameliorare la pubertate, sub androgeni

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1. Boala fon Willebrand- precursor F Willebrand sintetizat de endoteliu si megacariocite- functii F Willebrand

- adeziunea trombocit la endoteliu- transportor f VIII

- TS - prelungit- agregare plachetara la ristocetina= absenta - APTT– normal, T Quik-normal- concentratia de F Willebrand - scade

- concentratia plasmatica: 10 mg/dl

- tratament: crioprecipitat, desmopresin

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- Boala Von Willebrand - 1 la 100 – 500 persoane este cea

mai comuna anomalie mostenita de sangrare

- FvW - glicoproteina multimerica heterogena secretata de endoteliu si

plachete

1. favorizeaza adeziunea plachetelor la receptori subendoteliali

2. transportor plasmatic al F VIII- nivel palsmatica 10mg/L

– reduceri modeste FvW sau modificare moleculara determina S hemoragipar

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Boala von Willebrand:- boala autosomal dominanta, forma

heterozigota vWf este produs de gena normala

- BvW tip I - nivel descrescut cu 50% al FvW

- Clinic: sangerare la traumatisme, chirurgie, epistaxis, hemoragii mici mucoase, subcutanate

- Diagnostic: - TS > - nivel plasmatic vWF < - aglutinare la ristocetina< (activitate

biologica)- spectru normal al multimerilor la

electroforeza in agaroza

- activitatea F VIII scazuta

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- Boala von Willebrand: - BvW tip II - nivel noraml FvW

disfunctional pentru multimerii cu greutate moleculara mare si medie, fenomen detectat la electroforeza in agaroza

- BvW tip II a - deficienta a FvW cu greutate moleculara mare sau medie, mutatie in

domeniul A2 – deficienta de secretie sau proteoliza a multimerilor vWF, - FvW si F VIII – concentratii normale

- BvW tip II b – descrestere FvW cu greutate moleculara mare, legare inadecvata de

plachete si eliminare a Pl+FvW anormal, cu trombocitopenie secundara

- mutatie in domeniul I ce se laga de glicoproteina Ib IX

- - FvW si F VIII – concentratii

normale

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- BvW tip III - autosomal recesiva- incidenta 1 la 1 milion

- parinti heterozigoti pentru defecte diferite sau homozigoti pentru acelasi defect – FvW absent + F VIII redus

Clinic: sangerari de mucoase + hemartroze

- BvW tip IIn- defect legare FvW de F VIII

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- Clinic:

- TS - prelungit- Agregare plachetara la ristocetina= absenta - APTT– normal, T Quik-normal- Concentratia plasmatica F Willebrand - scade

- femei si barbati- hemoragii cutanate si mucoase

- Tratament: crioprecipitat, desmopresin

- forme:- usoare, medii, severe

- DG. paralinic:

Page 105: Sindroame hemoragipare 2016

- Tratament- concentrate de F VIII

- menoragia – contraceptive orale

- DDAVP sau desmopresina (vaspresina) – creste retentia de fluide – stimuleaza

eliberarea FvW- nu in tipul IIa !!!

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Deficienta factor XI

- Tratament – plasma proaspata inghetata o infuzie/zi

- transmitere autosomal recesiva - mai frecventa la evrei din Ashkenazi

- Clinic – sangerare posttraumatica - menometroragii

Page 107: Sindroame hemoragipare 2016

Deficienta de factor V, VII. X si II

- Tratament – plasma proaspata inghetata o infuzie/zi

- transmitere autosomal recesiva - rare

- Clinic – sangerare posttraumatica - menometroragii

Page 108: Sindroame hemoragipare 2016

Afibrinogenemia si disfibrinogenemia

- Tratament – plasma proaspata inghetata

- Clinic – sangerare - tromboze in disfibrinogenemie (rar)

- Paraclinic- afibrinogenemie: scaderea concentratie de fibrinogen

- disfibrinogenemie: - Timp protrombina prelungit - Timp tromboplastina prelungit

- Timp trombina prelungit

Page 109: Sindroame hemoragipare 2016

1. Deficienta dobandita a factorilor de coagulare

- vitamina K -- plante- sintetizata de flora intestinala

vit K

epoxidaza

reductaza

vit K epoxid

Anticoagulanti cumarinici

Page 110: Sindroame hemoragipare 2016

- Deficienta vitamina K influenteaza -- f II- f VII- f IX- f X

- proteina C si S

Page 111: Sindroame hemoragipare 2016

- Cauze deficienta vitamina K -- aport insuficient- antibioterapie per os- sindr biliar obstructive- f X- boli hepatocelulare grave- antagonisti de vit K

Page 112: Sindroame hemoragipare 2016

- Clinic -- sindrom hemoragipar functie de deficienta de vit K- forme usoare - severe

- Tratament - - plasma proaspata, sange proaspat - vit K parenteral – 10mg - restabileste depozitele hepatice in 8 -10 ore

- paraclinic -- TC – prelungit, T Quik - prelungit

Page 113: Sindroame hemoragipare 2016

- Cauze de sangerare in boli de ficat

- scade sinteza : fibrinogen, protrombina, fact V, VII, IX, X, - scade sinteza: proteina C,S,

antitrombina III - CID - vit K - scade absorbtia si metabol - monocit - macrofag

insuficient functional

- fibrinoliza sistemica primara - trombocitopenie

Page 114: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Sindrom clinicopatologic caracterizat prin formarea unor retele de fibrina intravascular.

Page 115: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Mecanism: -generarea necontrolata de trombina -expunerea la nivele patologice de FT -supresia mecanismelor anticoagulante fiz. -fibrinoliza anormala

Page 116: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Insuficienta organica multipla: -depozite de fibrina in vasele mici si medii -afectarea circulatiei:F,R, creier,plaman -ischemie -hemoliza intravasculara

Page 117: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Consumul trombocitelor si factorilor de coag. - sangerare difuza -fibrinoliza -PDF, d-Dimeri

Page 118: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

- infectii sistemice: stafilo, meningococ, B Gram neg

- sindr obstetricale: avort, eclampsie, dezlipire placenta - neoplazii: LAM, adenocarcinoame - hemoliza intravasculara- distructii tisulare: traumatisme, arsuri, etc- leziuni endoteliu: SHU, GNA, hemangiom cavernos- ciroza

Page 119: Sindroame hemoragipare 2016

Factori etiologici

XII a

PDF exces in microcirculatie

XII a

Tromboze

Fibrinoliza in excesActivare coagulare in microcirculatie

Sangerare difuza

XII a

PDF exces in microcirculatie

PDFcoagulare fibrinoliza

Page 120: Sindroame hemoragipare 2016

Tromboze Sangerare difuza

- neurologic : semne focar, convulsii, coma

- piele : ischemie focala, gangrena

- plaman : SDRA

- gastrointestinal : sangerare acuta

- laborator : trombocite <, F VII, V<, Fibrinogen<, PDF> 4g/dl, AH microangiopatica

- petesii,- sangerare - pe mucoase -(gastrointestinala, hemoptizieepistaxis) - neurologica

Page 121: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Page 122: Sindroame hemoragipare 2016

Coagulare intravasculara diseminata

Page 123: Sindroame hemoragipare 2016

CID – dg. paraclinic 1. trombocitopenie

2. AH microangiopatica

schizocite

3. TC >, aPTT >, TQ >

5. PDF+++4. Fibrinogen <

Page 124: Sindroame hemoragipare 2016

- Tratament

- tratamentul bolii de baza - reechilibrare hidroelectrolitica - heparina –1000 UI/ora, 5000 UI la 4ore - EACA = 4-6g/24 ore

( blocheaza PDF)

Page 125: Sindroame hemoragipare 2016

Coagulare intravasculara diseminatacronica

-hemangioame gigante-carcinoame metastatice-fetus mort

Page 126: Sindroame hemoragipare 2016

CID cronic– dg. paraclinic 1. Trombocite-N sau usor

2. AH microangiopatica

schizocite3. aPTT -N, TQ -N

5. PDF+++4. Fibrinogen -N

Page 127: Sindroame hemoragipare 2016

- Fibrinoliza

activator tisular plasminogen

plasminogen tromb

plasminafibrina

PDF

anti plasmina2

PAI

Page 128: Sindroame hemoragipare 2016

- Fibrinoliza primara

- deficitul anti plasmina2

- scade mult fibrinogenul

- sindrom hemoragipar

- aPTT, T Quick – aproape normale - trombocite normale

- paraclinic

- tratament - plasma proaspata, EACA

Page 129: Sindroame hemoragipare 2016

ELEMENTE CLINICE DIAGNOSTIC DIFERENTIAL

- varsta, sex - Sindromul hemoragipar

- conditii de declansare - purpura - localizare - leziuni palpabile, buloase,

micronecrotice - acuta, cronica

- hemoragii: mucoase, articulare, SNC

Page 130: Sindroame hemoragipare 2016

ELEMENTE PARACLINICE DIAGNOSTIC DIFERENTIAL

- Timp sangerare - Trombocite - Timp coagulare

- concentratie f VIII, IX, von willebrand

- aPTT - t Quick

- PDF - liza cheagului

- fibrinogen