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Coagulation

Editorial Board Members: Kyle Annen, D.O., Melissa R. George, D.O., Patricia Tsang, M.D., M.B.A., Mrigender Singh Virk, M.D.
Deputy Editor-in-Chief: Patricia Tsang, M.D., M.B.A.

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Editorial Board oversight: Melissa R. George, D.O. (last reviewed December 2022), Kyle Annen, D.O. (last reviewed February 2021), Patricia Tsang, M.D., M.B.A. (last reviewed September 2021)
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Superpage Topics
4 factor PCC Acquired thrombophilia - general Activated protein C resistance / Factor V Leiden Algorithm for workup of hereditary bleeding disorders D-dimer / dimerized plasmin fragment D Dabigatran Disseminated intravascular coagulation (DIC) Factor II (prothrombin) deficiency Factor VIII deficiency (hemophilia A) Factor XI deficiency Factor XII deficiency Factor XIII deficiency Heparin Heparin - low molecular weight Heparin induced thrombocytopenia Heparin induced thrombocytopenia (HIT) Hyperhomocysteinemia Mixing studies Physiology Protein C deficiency Viscoelastic hemostatic assays von Willebrand disease and testing
4 factor PCC
Diagrams / tables
None
Videos
None
Acquired thrombophilia - general
Clinical images

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Thrombi in major trunks of pulmonary artery

Activated protein C resistance / Factor V Leiden
Diagrams / tables

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Protein C pathway

Algorithm for workup of hereditary bleeding disorders
Diagrams / tables
Bleeding disorder Prevalence Inheritance pattern
Factor I (fibrinogen) deficiency
  • Afibrinogenemia
  • Hypofibrinogenemia
  • Dysfibrinogenemia
    		• More than 200 cases reported
    Autosomal recessive
    Autosomal dominant or recessive
    Autosomal dominant or recessive
    Factor II (prothrombin) deficiency Less than 100 cases reported Autosomal recessive
    Factor V deficiency Less than 1 in 1,000,000 Autosomal recessive
    Factor VII deficiency 1 in 500,000 Autosomal recessive
    Factor VIII deficiency 1 in 5000 male births X-linked recessive
    Factor IX deficiency 1 in 30,000 male births X-linked recessive
    Factor X deficiency 1 in 500,000 Autosomal recessive
    Factor XI deficiency 4% in Ashkenazi Jews, otherwise rare Autosomal recessive
    Factor XIII deficiency More than 200 cases reported Autosomal recessive
    Combined factor deficiencies
  • Factor V-Factor VIII
  • Factor II, VII, IX, X

    • > 30 families reported
    < 15 families reported
    Autosomal recessive
    Autosomal recessive
    a2-antiplasmin deficiency > 10 families reported Autosomal recessive
    a1-antitrypsin Pittsburgh deficiency Only 3 cases reported Autosomal dominant
    von Willebrand Disease (VWD)
  • Type I
  • Type II
  • Type III
    		• 1 in 100
    Autosomal dominant
    Autosomal dominant
    Autosomal recessive
    Glanzmann thrombasthenia 1 in 1,000,000 Autosomal recessive
    Bernard-Soulier syndrome < 1 in 1,000,000 Autosomal recessive
    Gray platelet syndrome Rare Autosomal dominant, recessive or X-linked recessive
    Wiskott-Aldrich syndrome 1 in 1,000,000 X-linked recessive


    Disorders of Primary Hemostasis
    von Willebrand disease
    Glanzmann thrombasthenia
    Bernard-Soulier syndrome
    Platelet storage pool disease
    Gray platelet syndrome
    Wiskott-Aldrich syndrome


    Disorders of Secondary Hemostasis
    Factor I (fibrinogen) abnormalities
  • Afibrinogenemia
  • Hypofibrinogenemia
  • Dysfibrinogenemia
    • Factor II (prothrombin) deficiency
    Factor V deficiency
    Factor VII deficiency
    Factor VIII deficiency (Hemophilia A)
    Factor IX deficiency (Hemophilia B)
    Factor X deficiency
    Factor XI deficiency
    Factor XIII deficiency
    Combined factor deficiencies
    a2-antiplasmin deficiency
    a1-antitrypsin deficiency
    Ehlers-Danlos syndrome
    Osler-Weber-Rendu syndrome
    Scurvy (vitamin C deficiency)
    D-dimer / dimerized plasmin fragment D
    Clinical images

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    Fibrin split products / D-dimer

    Dabigatran
    Diagrams / tables

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    Structure and site of inhibition in coagulation cascade

    Disseminated intravascular coagulation (DIC)
    Diagrams / tables

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    Clinical diagnosis / management of patients with DIC

     

    Key events in DIC




    Table 1: secondary causes
    Condition
    Examples
    Severe infectious diseases Gram positive or negative organisms, malaria, hemorrhagic fevers
    Malignancy Solid tumors (e.g. adenocarcinomas), acute promyelocytic leukemia or monocytic leukemia
    Trauma Multitrauma, brain injury, burns
    Obstetrical complications Abruptio placentae, amniotic fluid embolism
    Vascular malformations Kasabach-Merrit syndrome, giant hemangiomas
    Other vascular malformations, large aortic aneurysms
    Severe immunologic reactions Transfusion reaction
    Heat stroke
    Postcardiopulmonary resuscitation


    Table 2: DIC scoring systems by the JAAM and the ISTH (AMIA Annu Symp Proc 2015;2015:804)
    Japanese Association for Acute Medicine (JAAM)
    SIRS* criteria
    ≥ 3 +1
    0 to 2 0
    Platelet count
    < 80 × 109/L or > 50% decrease within 24 hours +3
    ≥ 80 < 120 × 109/L or > 30% decrease within 24 hours +1
    ≥ 120 × 109/L 0
    Prothrombin time (value of patient / normal value)
    ≥ 1.2 +1
    < 1.2 0
    Fibrin / fibrinogen degradation products
    ≥ 25 mg/L +3
    ≥ 10 < 25 mg/L +1
    < 10 mg/L 0
    Diagnosis: if ≥ 4, there is positive diagnosis of DIC
    *Systemic inflammatory response syndrome
     
    International Society of Thrombosis and Hemostasis (ISTH)
    Platelet count
    < 50 × 109/L +2
    ≥ 50 < 100 × 109/L +1
    ≥ 100 × 109/L 0
    Elevated fibrin related marker
    Strong increase +3
    Moderate increase +2
    No increase 0
    Prolonged prothrombin time
    ≥ 6 seconds +2
    ≥ 3 < 6 seconds +1
    < 3 seconds 0
    Fibrinogen level
    < 100 g/mL +1
    ≥ 100 g/mL 0
    Diagnosis: if > 5, there is positive diagnosis of overt DIC;
    if < 5, suggestive (not affirmative) of nonovert DIC
    Factor II (prothrombin) deficiency
    Diagrams / tables

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    Coagulation cascade

    Factor VIII deficiency (hemophilia A)
    Diagrams / tables

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    Coagulation cascade

    Factor XI deficiency
    Diagrams / tables

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    Coagulation cascade

    Factor XII deficiency
    Diagrams / tables

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    Coagulation cascade

     

    Mechanisms of factor XII activation

    Factor XIII deficiency
    Diagrams / tables

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    Diagram of factor XIII activation

    Heparin
    Diagrams / tables

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    Heparin 2-D structure

    Function of heparin

    Heparin - low molecular weight
    Diagrams / tables

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    Function of LMWH

    Heparin induced thrombocytopenia
    Diagrams / tables

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    4-T score chart

    Heparin induced thrombocytopenia (HIT)
    Diagrams / tables

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    Pathophysiology of HIT

    Hyperhomocysteinemia
    Diagrams / tables

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    Folate metabolism

    Mixing studies
    Diagrams / tables

    Contributed by Tori Seasor, M.D. and Karen A. Moser, M.D.
    Mixing study performance and interpretation

    Mixing study performance and interpretation



    Differential diagnosis of prolonged PT or aPTT incorporating mixing study results (correction versus noncorrection)
    Clotting time prolonged Mixing study result  Key differential diagnoses
    Prothrombin time (PT) Corrects
    • Factor deficiency (VII, X, V, II)
    • Warfarin
    • Vitamin K deficiency
    • Liver disease
    Does not correct
    • Specific factor inhibitor (VII, X, V, II)
    • Direct Xa inhibitor
    Activated partial
    thromboplastin time (aPTT)     		Corrects
    • Factor deficiency (VIII, IX, XI, contact factors)
    • Rarely, von Willebrand disease (if VIII is low enough to prolong aPTT)
    Does not correct
    • Specific factor inhibitor (VIII, IX, XI, contact factors)
    • Heparin
    • Direct thrombin inhibitor
    • Lupus anticoagulant
    PT and aPTT Corrects
    • Common pathway factor deficiency (X, V, II)
    • Warfarin (high doses) or superwarfarin
    • Vitamin K deficiency
    • Disseminated intravascular coagulation
    • Liver disease
    Does not correct
    • Common pathway factor inhibitors (X, V, II)
    • Direct thrombin inhibitors
    • Direct Xa inhibitors
    • Lupus anticoagulant (rarely)
    • Heparin (very high doses)
    Physiology
    Diagrams / tables

    Contributed by Zaher K. Otrock, M.D.

    Summary of secondary hemostasis



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    Coagulation

     

    Intrinsic pathway

    Thrombomodulin function

    Thrombomodulin protein

    Protein C deficiency
    Clinical images

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    Warfarin induced skin necrosis

    Viscoelastic hemostatic assays
    Diagrams / tables

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    Parameter tracing example

    Parameter tracing example

    Thromboelastography<br>trace patterns

    Thromboelastography
    trace patterns

    Rotational thromboelastometry trace patterns

    Rotational thromboelastometry trace patterns

    von Willebrand disease and testing
    Diagrams / tables

    Table 1: Clinical characteristics of vWD subtypes (Clin Appl Thromb Hemost 2017;23:900)
    Condition Inheritance pattern Common mutation domain Defect Clinical presentation
    Type 1 Autosomal dominant Throughout vWF gene (OMIM: Von Willebrand Disease, Type 1 [Accessed 3 August 2022]) Decreased concentration of functionally normal vWF Mild to moderate mucocutaneous bleeding
    Type 2A Autosomal dominant (OMIM: Von Willebrand Disease, Type 2 [Accessed 3 August 2022]) A1, A2 Enhanced susceptibility to cleavage by ADAMTS13 Moderate to severe mucocutaneous bleeding
    CK Impaired dimer assembly
    D1, D2 Impaired multimer assembly
    A1, A2, D3 Impaired secretion of vWF, with enhanced intracellular retention
    Type 2B Autosomal dominant (OMIM: Von Willebrand Disease, Type 2 [Accessed 3 August 2022]) A1 Increased affinity of GPIb binding site on vWF for the GPIb receptor Moderate to severe mucocutaneous bleeding
    Type 2M Autosomal dominant (OMIM: Von Willebrand Disease, Type 2 [Accessed 3 August 2022]) A1 Reduced affinity of GPIbα binding site on vWF for the GPIb receptor Moderate to severe mucocutaneous bleeding
    A3 Reduced affinity of vWF for collagen
    Type 2N Autosomal recessive (OMIM: Von Willebrand Disease, Type 2 [Accessed 3 August 2022]) D' - D3 Reduced affinity of vWF for FVIII Moderate to severe hemophilia-like bleeding
    Type 3 Autosomal recessive (OMIM: Von Willebrand Disease, Type 3 [Accessed 3 August 2022]) Throughout vWF gene Null alleles result in virtual absence of vWF Severe mucocutaneous bleeding and hemophilia-like bleeding
    Rare: D1 - D2 Impaired dimer formation and intracellular retention


    Table 2: Laboratory characteristics of subtypes of vWD (Clin Appl Thromb Hemost 2017;23:900, Teruya: Management of Bleeding Patients, 2nd Edition, 2021)
    Condition vWF antigen level (vWF:Ag) in % vWF activity (vWF:Act) in % vWF:Act / vWF:Ag Factor VIII (FVIII) Multimers
    Type 1 < 30 < 30 > 0.7 Low, normal All low
    Type 2A Mildly low Low < 0.7 Normal, mildly low Absent high and intermediate multimers
    Type 2B Normal, low Low < 0.7 Normal, mildly low Absent high multimers
    Type 2M Normal, low Low < 0.7 Normal, mildly low Normal
    Type 2N Normal, low Normal, low > 0.7 Low Normal
    Type 3 < 3 < 3 Not applicable Low Absent
    Low vWF 30 - 50 Normal, low > 0.7 Normal Normal
    Normal 50 - 200 50 - 200 > 0.7 Normal Normal
    Molecular / cytogenetics images

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    vWF multimer analysis

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