Plasmin

FactorXIIIa

Plasmin

Fibrinogen(Factor I)

FactorIIa

FactorIIa

FibrinPolymers

Ca++

Fibrinopeptide A

ActivatedPlatelets

FibrinMonomer

FDPsFibrinogen Degradation

ProductsFDPs & XDPs

(Cross-linked DPs)(e.g. D-dimers) Stable Thrombus

(Clot)

FactorIIa

Fibrinopeptide B

Factor XIIIFibrin Stabilizing Factor (FSF)

Biosynthesis: Megakaryocytes, liverMW: 320,000 daltonsPlasma Concentration: 10mg/LIn Vivo Half-Life: 12 daysPathology: FSF deficiency, autosomal recessive

SolubleFibrin

Polymer

FactorXIII

Activation Procoagulant

Antico

agul

ant

Activation

Procoagulant

Inhibition

Activatio

n

Activation

Procoagulant

Inhib

ition

Anticoa

gulan

tInhi

bitio

nInhibition

Anticoagulant

Anticoagulant

Plasmin

Fibrin(Factor Ia)

Procoagulant

Biosynthesis: LiverMW: 340,000 daltonsPlasma Concentration: 2500 mg/LIn Vivo Half-Life: 20 hoursPathology: Afibrinogenemia, autosomal recessive. Dysfibrinogenemia, autosomal dominant

FibrinogenFactor I

HighMolecular

WeightKininogen

Kinins

Inhibition

Activation

Procoagulant

Anticoagulant

Biosynthesis: LiverMW: 80,000 daltonsPlasma Concentration: 29 mg/LIn Vivo Half-Life: 60 hoursPathology: Hageman trait, autosomal recessive

Factor XIIHageman Factor

Factor XIIa

C1EsteraseInhibitor

FactorXII

NegativelyCharged

Activating Surface

Kalli-krein

*FXIIf alter vascular permeability

HMWK

High MolecularWeight

Kininogen (HMWK)Fitzgerald Factor

MW: 120,000 daltonsPlasma Concentration: 70 mg/LPathology: Fitzgerald trait,autosomal recessive

FactorXIIa

C1

Esteras

e

Inhibitor

(e.g., kaolin,ellagic acid

silica)

Factor XIIFragments*

High Blood Pressureand Inflammation

Procoagulant

Anticoagulant

Activation

Inhibition

HighMolecular

WeightKininogen

Ca++

Biosynthesis: LiverMW: 158,000 daltonsPlasma Concentration: 4 mg/LIn Vivo Half-Life: 65 hoursPathology: Hemophilia C, autosomal recessive

Factor XIPlasma ThromboplastinAntecedent

FactorXIa

FactorXIa

α1- Anti-

trypsin

Factor XI

FactorXIIa

α1-

Anti-

trypsin

Heparin

Biosynthesis: Liver, Vitamin K dependentMW: 55,000 daltonsPlasma Concentration: 5 mg/LIn Vivo Half-Life: 65 hours Pathology: Stuart disease, autosomal recessive

Factor XStuart-Prower Factor

FactorXa

Factor X

TissueFactor

FactorVIIa

Ca++

Procoagulant

FactorIXa

FactorXa

Heparin

Anti-thrombin

III

Anti-

thrombin

III

or

Ca++

Phospho-lipid (Platelet

Factor 3)

Anticoagulant

Tissue FactorFactor III, Thromboplastin

Biosynthesis: Brain, lung, subendotheliumMW: 45,000 daltons

ExtrinsicX-aseComplex

IntrinsicX-ase

Complex

FactorVIIIa

Activation

Inhibition

ProteinSPhospholipid

(Platelet Factor 3)

ActivatedProtein

C

Biosynthesis: Liver, endothelium; Factor VIII Related Antigen, megakaryocyteMW(FVIII + vWF): 1.2-2 million daltons (6-10 subunits – 200,000 daltons each)Plasma Concentration: 7 mg/L (vWF)In vivo Half-Life: 10 hours (Factor VIII)Pathology: Factor VIII-Hemophilia A, x-linked recessive. vWF-von Willebrand’s disease, autosomal dominant

Factor VIIIAntihemophilic Factor

Factor VIIIa

FactorVIII

FactorIIa

Anticoagula

nt

InactiveFragments

Ca++

FactorXa

Procoagulant

or

von Willebrand FactorvWF

Protein CComplex

Inhibition

Activation

TissueFactor

Biosynthesis: Liver, Vitamin K dependentMW: 55,000 daltonsPlasma Concentration: 1 mg/LIn Vivo Half-Life: 5 hoursPathology: Hypoproconvertinemia, autosomal recessive

Factor VIIProconvertin,Stable Factor

Factor VIIa

TFPI*

FactorVII

FactorXa

Phospholipid(Platelet Factor 3)

Ca++

Anticoagulant

FactorIXa

HighMolecular

WeightKininogen

Ca++

FactorXa

TFPI

*

FactorXIIa

or or

*TissueFactorPathwayInhibitor (a.k.a. LACIor EPI)

Inhibition

Activation

FactorVIIa

Procoagulant

ProteinSPhospholipid

(Platelet Factor 3)

ActivatedProtein

C

Biosynthesis: Liver, megakaryocytesMW: 330,000 daltonsPlasma Concentration: 5-12 mg/LIn Vivo Half-Life: 25 hoursPathology: Parahemophilia, autosomal recessive

Factor VProaccelerin,Labile Factor

Factor Va

FactorV

FactorIIa

Ca++

Anticoagulant

Procoagulant

InactiveFragments

Protein CComplex

Inhibition

Activation

Biosynthesis: Liver, Vitamin K dependentMW: 57,000 daltonsPlasma Concentration: 4 mg/LIn Vivo Half-Life: 20 hours Pathology: Hemophilia B, (Christmas disease) x-linked recessive

Factor IXChristmas Factor

FactorIXa

Factor IX

TissueFactor Factor

VIIa

Ca++

Procoagulant

Anticoagulant

FactorXIa

Heparin

FactorIXa Heparin

Anti-thrombin

III

or

Ca++

Activation

Inhibitio

n

Anti-

thrombin

III

Heparin

Biosynthesis: Liver, Vitamin K dependentMW: 70,000 daltonsPlasma Concentration: 100 mg/LIn Vivo Half-Life: 100 hoursPathology: Hypoprothrombinemia, autosomal recessive

Factor IIProthrombin

FactorIIa

Factor IIa(Thrombin)

HeparinAnti-thrombin

III

FactorII

FactorVa

Phospholipid(Platelet Factor 3)

FactorXa

Ca++

Anticoagulant

ActivationFragments

Prothrombinase Complex

Procoagulant

Anti-

thrombin

III

Activation

Inhibition

*

*or HeparinCofactor II

Thrombo-modulin

FactorIIa

Biosynthesis: Liver, Vitamin K dependentMW: 56,000 daltonsPlasma Concentration: 3-5 mg/LIn Vivo Half-Life: 6-7 hours Pathology: Protein C deficiency, autosomal recessive (?)

Protein C

ActivatedProtein

C*

ActivatedProtein C

Protein C

Protein CInhibitor

Procoagulant

Anticoagulant

Protein CActivation

Peptide

* Requires Protein S for functional activity

Activatio

n

Inhibition

Protein CInhibitor

Kalli-krein

Uro-kinaset-PA

Fibrinolysis

IntrinsicPathway(aPTT)

ExtrinsicPathway

(PT)

Va

X

IIa Plas-minogen

XI

XII

Pro-UrokinaseIX

TissueFactor

VIIa

VII

Kinins

IXa

XIa

XIIa

Prekalli-krein

HMWK

VIIIa

VIII

XaX

II

V

Fibrinogen

CommonPathway

Fibrin Clot+ Platelet Plug

XIIIa

FibrinPolymer

XIII

Plasmin

tPA(tissue

PlasminogenActivator) Antic

oagulant

Procoagulant

Activation

Inhibition

Biosynthesis: LiverMW: 90,000 daltonsPlasma Concentration: 120 mg/LIn Vivo Half-Life: 48 hoursPathology: Plasminogen deficiency, autosomal dominant. Dysplasminogenemia, autosomal recessive

Plasminogen

Plasmin

Plasmin

α2 - Anti-plasmin

Plasmin-ogen

Urokinase

α2 - Anti-

plasmin

Kallikrein

or or

Ca++

Biosynthesis: Probably LiverMW: 107,000 daltonsPlasma Concentration: 50 mg/LPathology: Fletcher trait, autosomal recessive

PrekallikreinFletcher Factor

Activation

Inhibition

HighMolecular

WeightKininogen

Kallikrein

Prekalli-krein

FactorXIIa

Procoagulant

Anticoagulant

C1 EsteraseInhibitor

C1

Esteras

e

Inhibitor

Kalli-krein

HemostasisPathways

This wall chart prepared by:David A. Barrow, Ph.D.Helena Laboratories, Beaumont, TX

In cooperation with:H. James Day, M.D.A. Koneti Rao, M.D.Alvin H. Schmaier, M.D.Temple University Hospital, Philadelphia, PA

Helen Ridgway, Ph.D.Wadley Institutes, Dallas, TX

Gordon E. Ens, MT(ASCP)Colorado Coagulation Consultants, Denver, CO

John Lazarchick M.D.Medical University of South Carolina, Charleston, SC

586-1112/91(3)

For information on Helen's complete line of Hemostasisproducts, call

Toll Free 800-231-5663

LaboratoriesHelena HemostasisSystems

PO BOX 752BEAUMONT TX 77704-0752

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