Thrombophilia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Asiri Ediriwickrema, M.D., M.H.S. [2]

Overview

Thrombophilias are classified as either inherited or a primary hypercoagulable state, acquired or a secondary hypercoagulable state, or mixed/unknown. Certain conditions are associated with greater thrombotic risks and both venous and arterial clots.

Classification

  • Thrombophilia can be classified in various forms[1].
  • Inherited thrombophilia or primary hypercoagulable state
  • Acquired thrombophilia or secondary hypercoagulable state
  • Mixed/Unknown
  • Venous verse arterial clots
Thrombophilia Classification
Inherited (Primary) Acquired (Secondary) Mixed/Unknown
Activated protein C (APC) resistance (Factor V Leiden) Age Hyperhomocysteinemia
Prothrombin gene mutation (Prothrombin G20210A) Immobilization APC resistance unrelated to Factor V Leiden.
Antithrombin deficiency Trauma/major surgery Increased factor VIII level
Protein C and protein S deficiency Orthopedic surgery Increased factor XI level
Dysfibrinogenemia Malignancy Increased factor IX level
Myeloproliferative disorders (polycythemia vera, essential thrombocythemia, hyperviscosity) Increased levels of thrombin-activatable fibrinolysis inhibitor (TAFI)
Pregnancy Decreased levels of free tissue factor pathway inhibitor (TFPI)
Estrogen and testosterone (oral contraceptives and hormone replacement therapy)
Obesity
Heart Failure
Cirrhosis
Chronic renal disease (nephrotic syndrome)
Antiphospholipid syndrome (APLS) or lupus anticoagulant
Heparin induced thrombocytopenia (HIT)
Disseminated intravascular coagulopathy (DIC)
Paroxysmal nocturnal hemoglobinuria (PNH)
Vasculitis
Thrombotic microangiopathy
Drug related (chemotherapies including L-aspariginase, mitomycin, infusion of clotting factors including prothrombin complex concentrates, cryoprecipitate)
Thrombophilic states associated with arterial clots
APLS and lupus anticoagulant
HIT
DIC
PNH
Cold agglutinins (associated with mycoplasma infections)
Vasculitis
Hyperhomocysteinemia
Thrombophilic state Thrombotic risk
Trauma/General surgery Modest
Age > 60 Modest
Immobilization Modest
Pregnancy Modest
Hormone therapies Modest
Factor V Leiden Modest
Prothrombin mutation Modest
Homocysteinemia Modest
Protein C and S deficiency Intermediate
Malignancy High
APLS/Lupus anticoagulant High
Myeloproliferative disorders/hyperviscosity High
PNH High
Orthopedic surgery High
Antithrombin deficiency High
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].


  • The most common classification is by the nature of the thrombosis: arterial, venous or combined.

References

  1. Hoffman R, Benz EJ, Shattil SJ, et al. Hematology: Basic Principles and Practice: Elsevier Science Health Science Division; 2004.

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