Αιματολογία Ι - Θ

Αιματολογία Ι - Θ

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. 2 ( 5 ) 1. . 2. . 3. . 4. . 5. . 3 - , . . 4

. , () . Endotelijalna elija, Aleksa Lukic CC BY-SA 3.0 5 1/2 PAI (Plasminogen activator inhibitor), t-PA. Von Willebrand (vWF) FVIII. , . tissue factor TF. 6

2/2 (PGI2)) . (heparin sulfates) , . (thrombomodulin) C. (nitrous oxide, NO)), . t-PA (tissue plasminogen activator), . 7 TF vWF PAI-1 P-selectin

TPA TFPI 8 Blausen 0649 Monocyte, CFCF CC BY 3.0 Platelets by budding off from megakaryocytes, CC BY-SA 3.0 9 1/3 , , .

Platelet structure, Graham Beards CC BY-SA 3.0 10 2/3 Platelets2, Graham Beards CC BY-SA 3.0 11 3/3 haematologica.org 12 1/17 , , . .

13 2/17 Name Description Molecular Weight (MW) = Fibrinogen (Factor 1) 340,000 daltons (Da); glycoprotein Prothrombin (Factor MW = 72,000 Da; vitamin KII) dependent serine protease Tissue factor (Factor MW = 37,000 Da; also known III) as thromboplastin of Ca++ ions for Calcium ions (Factor Necessity coagulation reactions IV) described in 19th century Factor V (Labile MW = 330,000 Da factor) Function Adhesive protein that forms the fibrin clot

Activated form is main enzyme of coagulation Lipoprotein initiator of extrinsic pathway Metal cation necessary for coagulation reactions Cofactor for activation of prothrombin to thrombin . 14 3/17 Name FactorVII (Proconvertin) Factor VIII (Antihemophilic factor) Factor IX (Christmas factor) Description MW = 50,000 Da; vitamin Kdependent serine protease

Function With tissue factor, initiates extrinsic pathway MW = 330,000 Da Cofactor for intrinsic activation of factor X Factor X (StuartPrower factor) MW = 58,900 Da; vitamin Kdependent serine protease MW = 55,000 Da; vitamin Kdependent serine protease Activated form is enzyme for intrinsic activation of factor X Activated form is enzyme for final common pathway activation of prothrombin 15 4/17 Name

Description Factor XI (Plasma MW = 160,000 Da; serine thromboplastin protease antecedent) Factor XII (Hageman MW = 80,000 Da; serine factor) protease Factor XIII (Fibrin MW = 320,000 Da stabilizing factor) High-molecularweight kininogen MW = 1 10,000 Da; circulates (Fitzgerald. Flaujeac, in a complex with factor XI or William factor) Function Prekallikrein (Fletcher factor) Activated form that participates at beginning of aPTT-based intrinsic pathway

MW = 85,000 Da; serine protease Activated form is intrinsic activator of factor IX Factor that nominally starts aPTT-based intrinsic pathway Transamidase that cross-links fibrin clot Cofactor 16 5/17 Name Description Function Molecular Weight (MW) protein that Fibrinogen (Factor 1) = 340,000 daltons (Da); Adhesive forms the fibrin clot glycoprotein MW = 72,000 Da; form is main

Prothrombin (Factor II) vitamin K-dependent Activated enzyme of coagulation serine protease MW = 37,000 Da; also Lipoprotein initiator of Tissue factor (Factor III) known as extrinsic pathway thromboplastin Necessity of Ca++ ions Metal cation necessary Calcium ions (Factor IV) for coagulation reactions described in 1 for coagulation reactions 9th century - IV . 17 6/17 Name Description = 58,900 Da; Factor X (Stuart-Prower MW vitamin K-dependent

factor) serine protease Function Cofactor for activation of prothrombin to thrombin With tissue factor, initiates extrinsic pathway Cofactor for intrinsic activation of factor X Activated form is enzyme for intrinsic activation of factor X Activated form is enzyme for final common pathway activation of prothrombin Factor XI (Plasma thromboplastin antecedent) Activated form is intrinsic activator of factor IX

Factor V (Labile factor) MW = 330,000 Da MW = 50,000 Da; Factor VII (Proconvertin) vitamin K-dependent serine protease FactorVIII (Antihemophilic factor) MW = 330,000 Da MW = 55,000 Da; Factor IX (Christmas vitamin K-dependent factor) serine protease MW = 1 60,000 Da; serine protease 18 7/17 Name Factor XII (Hageman factor) Description Function Factor that nominally

MW = 80,000 Da; serine starts aPTT-based protease intrinsic pathway Transamidase that crossMW = 320,000 Da links fibrin clot Factor XIII (Fibrin stabilizing factor) High-molecular-weight MW = 1 10,000 Da; kininogen (Fitzgerald, circulates in a complex Cofactor Flaujeac, or William with factor XI factor) Activated form that Prekallikrein (Fletcher MW = 85,000 Da; serine participates at beginning factor) protease of aPTT-based intrinsic pathway 19 8/17 Name

Description Function Molecular Weight (MW) = Adhesive protein that Fibrinogen (Factor 1) 340,000 daltons (Da); forms the fibrin clot glycoprotein = 72,000 Da; vitamin K- Activated form is main Prothrombin (Factor II) MW dependent serine protease enzyme of coagulation = 37,000 Da; also known Lipoprotein initiator of Tissue factor (Factor III) MW as thromboplastin extrinsic pathway Tissue Factor (TF) ( ) ( ) 20 9/17 Name

Description Function Molecular Weight (MW) = Adhesive protein that Fibrinogen (Factor 1) 340,000 daltons (Da); forms the fibrin clot glycoprotein = 72,000 Da; vitamin K- Activated form is main Prothrombin (Factor II) MW dependent serine protease enzyme of coagulation = 37,000 Da; also Lipoprotein initiator of Tissue factor (Factor III) MW known as thromboplastin extrinsic pathway Necessity of Ca++ ions for Metal cation necessary Calcium ions (Factor IV) coagulation reactions described in 1 9th century for coagulation reactions Cofactor for activation of Factor V (Labile factor) MW = 330,000 Da prothrombin to thrombin With tissue factor, MW = 50,000 Da;

vitamin KFactor VII (Proconvertin) dependent serine protease initiates extrinsic pathway 21 10/17 Name Description FactorVIII (Antihemophilic factor) MW = 330,000 Da Factor IX (Christmas factor) Function Cofactor for intrinsic activation of factor X form is enzyme MW = 55,000 Da; vitamin K- Activated intrinsic activation of dependent serine protease for factor X Activated form is enzyme Factor X (Stuart-Prower MW = 58,900 Da; vitamin K- for final common factor)

dependent serine protease pathway activation of prothrombin Factor XI (Plasma MW = 1 60,000 Da; serine Activated form is intrinsic thromboplastin protease activator of factor IX antecedent) 22 11/17 Name Description Factor XII (Hageman factor) MW = 80,000 Da; serine protease Factor XIII (Fibrin stabilizing factor) High-molecular-weight kininogen (Fitzgerald,

Flaujeac, or William factor) MW = 320,000 Da Prekallikrein (Fletcher factor) Function Factor that nominally starts aPTT-based intrinsic pathway Transamidase that crosslinks fibrin clot MW = 1 10,000 Da; circulates in a complex with Cofactor factor XI MW = 85,000 Da; serine protease Activated form that participates at beginning of aPTT-based intrinsic pathway 23

12/17 Name Description Function Molecular Weight (MW) protein that Fibrinogen (Factor 1) = 340,000 daltons (Da); Adhesive forms the fibrin clot glycoprotein MW = 72,000 Da; form is main Prothrombin (Factor II) vitamin K-dependent Activated enzyme of coagulation serine protease MW = 37,000 Da; also Lipoprotein initiator of Tissue factor (Factor III) known as extrinsic pathway thromboplastin - -.

24 13/17 Name Description Function Necessity of Ca++ ions Metal cation necessary Calcium ions (Factor IV) for coagulation reactions described in 1 for coagulation reactions 9th century Cofactor for activation of Factor V (Labile factor) MW = 330,000 Da prothrombin to thrombin MW = 50,000 Da; With tissue factor, Factor VII (Proconvertin) vitamin K-dependent initiates extrinsic serine protease pathway FactorVIII Cofactor for intrinsic MW = 330,000 Da (Antihemophilic factor) activation of factor X

MW = 55,000 Da; Activated form is enzyme Factor IX (Christmas vitamin K-dependent for intrinsic activation of factor) serine protease factor X Activated form is enzyme MW = 58,900 Da; Factor X (Stuart-Prower vitamin K-dependent for final common factor) pathway activation of serine protease prothrombin 25 14/17 Name Factor XI (Plasma thromboplastin antecedent) Description

Function MW = 1 60,000 Da; serine protease Activated form is intrinsic activator of factor IX Factor XII (Hageman factor) that nominally MW = 80,000 Da; serine Factor starts aPTT-based protease intrinsic pathway Transamidase that crossMW = 320,000 Da links fibrin clot Factor XIII (Fibrin stabilizing factor) High-molecular-weight MW = 1 10,000 Da; kininogen (Fitzgerald, circulates in a complex Cofactor Flaujeac, or William with factor XI

factor) Activated form that Prekallikrein (Fletcher MW = 85,000 Da; serine participates at beginning factor) protease of aPTT-based intrinsic pathway 26 : - -, . a (activated ) . 27 15/17 Name Description

Function Molecular Weight (MW) protein that Fibrinogen (Factor 1) = 340,000 daltons (Da); Adhesive forms the fibrin clot glycoprotein MW = 72,000 Da; form is main Prothrombin (Factor II) vitamin K-dependent Activated enzyme of coagulation serine protease MW = 37,000 Da; also Lipoprotein initiator of Tissue factor (Factor III) known as extrinsic pathway thromboplastin V VIII XIII . 28 16/17 Name

Description Function Necessity of Ca++ ions Metal cation necessary Calcium ions (Factor IV) for coagulation reactions described in 1 for coagulation reactions 9th century Cofactor for activation of Factor V (Labile factor) MW = 330,000 Da prothrombin to thrombin MW = 50,000 Da; With tissue factor, Factor VII (Proconvertin) vitamin K-dependent initiates extrinsic serine protease pathway FactorVIII Cofactor for intrinsic MW = 330,000 Da (Antihemophilic factor) activation of factor X MW = 55,000 Da; Activated form is enzyme Factor IX (Christmas vitamin K-dependent for intrinsic activation of factor) serine protease

factor X Activated form is enzyme MW = 58,900 Da; Factor X (Stuart-Prower vitamin K-dependent for final common factor) pathway activation of serine protease prothrombin 29 17/17 Name Factor XI (Plasma thromboplastin antecedent) Description Function MW = 1 60,000 Da; serine protease

Activated form is intrinsic activator of factor IX Factor XII (Hageman factor) that nominally MW = 80,000 Da; serine Factor starts aPTT-based protease intrinsic pathway Transamidase that crossMW = 320,000 Da links fibrin clot Factor XIII (Fibrin stabilizing factor) High-molecular-weight MW = 1 10,000 Da; kininogen (Fitzgerald, circulates in a complex Cofactor Flaujeac, or William with factor XI factor) Activated form that Prekallikrein (Fletcher MW = 85,000 Da; serine participates at beginning factor) protease of aPTT-based intrinsic

pathway 30 Name Description Tissue factor pathway Molecular Weight (MW) inhibitor (TFPI) = 33,000 daltons (Da) MW = 62,000 Da; Protein C vitamin K-dependent serine protease MW = 75,000 Da; Protein S vitamin K-dependent protein Antithrombin MW = 58,000 Da Function Inhibits the tissue

factor/factor Vila complex Activated form cleaves coagulation cofactors Va and Villa Cofactor for protein C Serpin that directly inhibits several of the serine proteases; requires heparin as a cofactor 31 , . , , () . , . : o (tPA), . o (uPA), .

o , , 32 (HMWK) . 1/2 o (Vasoconstriction) o o o ( ). Platelet activation-adhesion-aggregation. (Fibrin meshwork (clot)) ( , ) . Clot dissolution (fibrinolysis). 33 2/2

() () () () () () 34 . ( ). o Platelet activation. o , adhesion. o , aggregation. 35

- , , NO (PGI2). , . 36 1/4 AM , Von Willebrand (VWF). GP IIb/IIIa Ib (GPIb) .

GPIb GP Ib vWF usmle287.wordpress.com 37 2/4 - ..(footnote ( ). : vWF, FV, FXIII, (FI), (PAI) - (ADP), . 2) . 38

3/4 - ADP 2 (Tx2)) . 39 4/4 , () Von Willebrand . GPIIb/IIIa GPIb Von Willebrand . . 40

( ). . 41 1/3 () . . 42 2/3 K XII

XIIa VIIa XI VII XIa IX VIII X IXa Xa PL VIIIa

Va Ca++ () (a) PL: XIII

(TF) XIIIa 43 3/3 : - - 44 1/10 ( ). XII VII

XIIa TF XIa XI HMWK/Prekallikrein IX Ca VIIa ++ IXa VIII VIIIa

X Xa II Ca++ V Va XIII IIa I (soluble)soluble)) (soluble)soluble)) XIIIa (soluble)insoluble))

45 2/10 XII VII XIIa TF XIa XI VIIa HMWK/Prekallikrein IX

IXa Ca++ VIII VIIIa X Xa Ca++ V Va XIII

II IIa I (soluble)soluble)) (soluble)soluble)) XIIIa (soluble)insoluble)) 46 3/10 XII VII XIIa XI

TF XIa HMWK/Prekallikrein IX IXa VIIa Ca++ VIII VIIIa X Xa II Ca++ V

Va XIII IIa I (soluble)soluble)) (soluble)soluble)) XIIIa (soluble)insoluble)) 47 4/10 ( ). , () .

. (XIIa) , . Xla (Ca++). VIII ( ). , lX Xa. 48 5/10 XII VII XIIa XI TF XIa

HMWK/Prekallikrein IX IXa VIIa Ca++ VIII VIIIa X Xa II Ca++ V Va

XIII IIa I (soluble)soluble)) (soluble)soluble)) XIIIa (soluble)insoluble)) 49 6/10 (tissue factor-TF) . TF , , . , TF, VII (FVII), ( TF/ FVIIa).

TF/FVIIa : ) X Xa ) IX Ia. 50 7/10 XII VII XIIa XI TF XIa HMWK/Prekallikrein IX

IXa VIIa Ca++ VIII VIIIa X Xa II Ca++ V Va XIII IIa

I (soluble)soluble)) (soluble)soluble)) XIIIa (soluble)insoluble)) 51 8/10 FXa FVa, , (KAI : Va.,Xa, Ca++, ) . FXIII, . , , , a

. 52 9/10 XII VII XIIa XI TF XIa HMWK/Prekallikrein IX

IXa VIIa Ca++ VIII VIIIa X Xa Ca++ V Va II XIII IIa

I (soluble)soluble)) XIIIa (soluble)insoluble)) 53 10/10 o , o XI, o FVIII von Willebrand (vWF) , o V, , o XIII. 54

1/3 . 2 , . 55 2/3 . 1. , o (AT), o (Tissue Factor Pathway Inhibitor) TFPI. , IXa, Xa, XIa, 1:1. TFPI ( ). 56

3/3 2. C (PC), , S (PS) ( ). PC / () . H PC (APC) PS Va VIIIa . 57 C (PC) - S (PS) XII VII XIIa XI TF XIa

HMWK/Prekallikrein IX aPC IXa VIIa Ca++ VIII VIIIa PS X aPC

Xa Ca++ V Va XIII PS II IIa I (soluble)soluble)) XIIIa (soluble)insoluble))

(soluble)soluble)) 58 Copyright , 2014. . (). 9: - . : 1.0. 2014. : ocp.teiath.gr. Creative Commons , 4.0 [1] , . .. , ..., . . . [1] http://creativecommons.org/licenses/by-nc-sa/4.0/ :

, (.. ) , . CC-BY CC-BY-SA CC-BY-ND CC-BY-NC CC-BY-NC-SA

CC-BY-NC-ND CC0 Public Domain , . . , . . . . . . . . . , , . ,

, . . : ( ) . o o . .

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