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
- Inherited thrombophilia or primary hypercoagulable state
- Acquired thrombophilia or secondary hypercoagulable state
- Mixed/Unknown
- Different thrombophilic states are associated with venous or both venous and 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 levels |
Protein C and protein S deficiency | Orthopedic surgery | Increased factor XI levels |
Dysfibrinogenemia | Malignancy | Increased factor IX levels |
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, hormone replacement therapy, and selective estrogen receptor modulator) | ||
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) | ||
Autoimmune disorders (Vasculitis, celiac's disease, inflammatory bowel disease) | ||
Thrombotic microangiopathy | ||
Sickle cell disease | ||
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[3] |
---|---|
Trauma/General surgery | Modest |
Age > 60 | Modest |
Immobilization | Modest |
Pregnancy | Modest |
Hormone therapies | Modest |
Factor V Leiden heterozygosity | Modest |
Prothrombin mutation | Modest |
Homocysteinemia | Modest |
Increased factor VIII levels | Modest |
Increased factor IX levels | Modest |
Increased factor XI levels | Modest |
Protein C and S deficiency | Intermediate |
Dysfibrogenemia | Intermediate |
Malignancy | High |
APLS/Lupus anticoagulant | High |
Myeloproliferative disorders/hyperviscosity | High |
PNH | High |
Orthopedic surgery | High |
Antithrombin deficiency | High |
Factor V Leiden homozygosity | High |
References
- ↑ Hoffman R, Benz EJ, Shattil SJ, et al. Hematology: Basic Principles and Practice: Elsevier Science Health Science Division; 2004.
- ↑ Cohoon KP, Heit JA (2014). "Inherited and secondary thrombophilia". Circulation. 129 (2): 254–7. doi:10.1161/CIRCULATIONAHA.113.001943. PMC 3979345. PMID 24421360.
- ↑ Bates SM, Ginsberg JS (2004). "Clinical practice. Treatment of deep-vein thrombosis". N Engl J Med. 351 (3): 268–77. doi:10.1056/NEJMcp031676. PMID 15254285.