patologia hematologica acuta
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Patologia hematologică Patologia hematologică acută la copiiacută la copii
Valentin Ţurea
Doctor habilitat în medicină, Profesor universitar
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Tipurile de sângerare
Peteşial – macular Hematom Mixt Vascular – purpural Angiomatos
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Tromboplastina tisulară Stimulatori ai funcţiilor trombocitare:
adrenalină, noradrenalină, acid difosforic Asigură activarea prin contact a
trombocitelor şi a factorului XII Sintetizează factorul von Willebrand şi alţi
factori de coagulare
Endoteliul vascular:
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Endoteliul vascular:
Sintetizează şi eliberează Tromboxan A2 Eliberează Prostaciclina Produce activatorul tisular al fibrinolizei Asigură imposibilitatea activării prin contact Formează potenţialul anticoagulant
Heparină + Antitrombină III Menţine dzeta-potenţialul
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Funcţiile trombocitare
Angiotrofică Adezivitate Agregare Sinteză, acumulare şi eliberare Contractibilitate
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Funcţiile trombocitare
Funcţia angiotrofică – determină integritatea peretelui vascular prin menţinerea elasticităţii şi neadmiterea fragilităţii.
Agregarea şi adezivitatea Elimenarea factorilor trombocitari Formarea trombului primar Asigură retracţia cheagului Asigurarea participării în procesul de coagulare
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Factorii trombocitari
P1 – acceleratorul trombocitar, identic factorului V de coagulare
P2 – acceleratorul trombinei P3 – tromboplastina trombocitară, tromboplastina
parţială P4 – factor antiheparinic P5 – factor de coagulare, identic fibrinogenului P6 – trombostenina P9 – factor fibrinstabilizant P10 – serotonina P11 - ADP
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Testele :
Vasele – fragilitatea, rezistenţa capilară Proba Duke Proba Aivi Proba Şiticov Numărul trombocitelor Dimensiunile trombocitelor Aprecierea adeziunii şi agregării Reacţia de retracţie a cheagului
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Factorii plasmatici de coagulare
Fibrinogenul Protrombina Tromboplastina tisulară Ionii de Ca++ Proaccelerina, factorul labil Proconvertina, factorul stabil Globulina antihemofilică Tromboplastina plasmatică (factorulCristmas) Protrombinaza Predecesorul tromboplastinei plazmatice Factorul de contact, f. Hageman Factorul fibrinstabilizant, fibrinaza
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TFTF, VIIa, VIIa IXa, IXa, VIIIaVIIIa XIa, HMWKXIa, HMWK
XII, HMWK,XII, HMWK,prekallikreinprekallikrein
Xa, Xa, VaVa
ThrombomodulinThrombomodulinTHROMBINTHROMBIN
IX--IX-->IXa>IXa
X-->XaX-->Xa
II-->IIaII-->IIa
X-->XaX-->Xa
XI-->XIaXI-->XIaIX-->IXaIX-->IXa
ExtrinsicExtrinsicIntrinsicIntrinsic
PC-->APCPC-->APCII FibrinFibrin
monomermonomerFibrinFibrinpolymerpolymer
Fibrin IIFibrin II Cross-linkedCross-linkedclotclot
XIIIXIII
XIIIaXIIIa
FPAFPA FPBFPB
PlasminPlasmin
D-dimers,D-dimers,FDPFDP
Frag B,XFrag B,X
VIII-->VIIIaVIII-->VIIIa
PAI-1PAI-1
TFPITFPI Protein CProtein C
HS/ATIIIHS/ATIII
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Leucemiile Acute Limfoblastice
Nr Varianta LAL Caracteristicile de bază
1. Varianta Limfoblastică L1
Celulele limfoblastice sunt mici, citoplasma – redusă, cromatina – omogenă, nucleolii sunt mici şi nu în toate celulele. Pronosticul cel mai favorabil.
2. Varianta Limfoblastică L2
Celulele sunt de diverse dimensiuni, cu predominarea celulelor mari, cromatina – neomogenă, nucleolii – unul sau cîţiva mari, citoplasma – pronunţată, în unele cazuri bazofilă. Pronosticul mai puţin favorabil.
3. Varianta Limfoblastică L3
Celulele sunt mari, citoplasma – pronunţată, bazofilie cu vacuolizare, se observă un nucleol mare.
Pronosticul nefavorabil.
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Leucemiile aucte nelimfoblastice
Varianta
Leucemiei Acute
Varianta
morfologică
Caracteristicile de bază
Leucemie Acută
Mieloblastică fără maturaţie M1 Predomină celule mieloblastice, promielocitele <3%
Leucemie Acută Mieloblastică
cu semne de maturaţie M2 Celule mieloblastice, promielocite >3%
Leucemie Acută Promielocitară M3 Celule blastice de tip promielocitar cu granulaţii în citoplasmă >30%.
Leucemie Acută Mielomonoblastică
M4 Celule mieloblastice ≥20% şi
Celule monoblastice ≥20%.
Leucemie Acută Monoblastică M5a Celule blastice fără maturaţie, promielocite/monocite <3%.
Leucemie Acută Monoblastică M5b Celule blastice cu semne de maturaţie, promielocite/monocite >3%.
Eritroleucemie M6 Eritrocariocite >30% şi >10% eritroblaşti cu multe anomalii.
Leucemie Acută Megacarioblastică
M7 Predomină megacarioblaşti şi micromegacariocite, se poate asocia cu mielofibroza acută.
Leucemie Acută Nediferenţiată M0 Se identifică în baza criteriilor citochimice.
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Vă mulţumesc pentru atenţie!
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?
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Sindromul PHACE:
P - malfomaţiile cerebrale în fosa posterioară (posterior fossa brain malformations);
H - hemagioame (hemagiomas);
A - anomalii arteriale şi coractaţie de aortă (arterial anomalias and corctation of the aorta);
C - malformaţii cardiace (cardiac defects);
E - anomalii oculare (eye abnormalities).