II. History: Findings suggestive of Hypercoagulable State
- Thrombosis at a young age (age under 50 years)
- Family History of thrombosis
- Recurrent Thrombosis
- Thrombosis in an unusual site (hepatic, mesenteric, or cerebral)
- Pregnancies complicated by frequent Miscarriage or IUGR
- Idiopathic venous thrombembolism or unprovoked VTE (No known predisposing factors)
III. Etiologies: Primary Hypercoagulable States (Hereditary)
- Common Causes
- Factor V Leiden Defect
- Prothrombin 20210
- Homocystinuria or Hyperhomocysteinemia
- Uncommon Causes
IV. Etiologies: Secondary or Acquired Hypercoagulable States
- Antiphospholipid Antibody Syndrome (most common)
- Pregnancy
- Surgery
- Trauma
- Infection or Sepsis
- Malignancy
- Cancer in idiopathic Hypercoagulability with unprovoked VTE: 3.9% (as high as 20% in some studies)
- Basic evaluation for occult malignancy
- Standard interval HME screening is appropriate (e.g. Pap Smear, Mammogram, Colonoscopy)
- However CT Abdomen and Pelvis is not recommended for unprovoked VTE alone
- Medications
- Estrogen sources
- Hydralazine
- Phenothiazines
- Procainamide
- Myeloproliferative disorder
- Hyperlipidemia
- Homocystinuria
- Lupus Inhibitor
- Nephrotic Syndrome
V. Labs: Indications for testing based on level of suspicion
-
General
- Hold blood for tests below before Anticoagulation
- Risk of Thrombophilia determines level of testing
- Unlikely to be thrombophilic
- Criteria
- First episode of Venous Thromboembolism and
- Known Thromboembolism Risk Factors and
- No Family History of Thromboembolism
- No first degree relative under age 50 years
- Testing
- No testing indicated
- Criteria
- Weakly thrombophilic
- Criteria
- Age over 50 years and
- First thromboembolic episode and
- No known Thromboembolism Risk Factors
- No Family History of Thromboembolism
- No first degree relative under age 50 years
- Testing
- Evaluation of common causes (see below)
- Criteria
- Strongly thrombophilic
- Criteria
- Age <50, no known Thromboembolism Risk Factors or
- Recurrent thromboembolic episode or
- Family History of Thromboembolism
- First degree relative under age 50 years
- Testing
- Evaluation of common causes and
- Evaluation of less common causes (see below)
- Criteria
- Unlikely to be thrombophilic
VI. Labs: Evaluation of common causes ($250)
- Complete Blood Count with platelets and morphology
- Prothrombin Time
-
Partial Thromboplastin Time
- Lupus Anticoagulant Syndrome suspected if increased PTT that does not correct with 1:1 dilution with normal plasma
-
Connective Tissue Disorder tests
- Antinuclear Antibody Test (ANA)
- Antiphospholipid Antibody Tests
- Clotting Assay for Lupus Anticoagulant (affected by Anticoagulants)
- ELISA for Anticardiolipin Antibodies
- B2 Glycoprotein Antibody may ultimately replace these tests as it has higher Specificity
- Test 3-4 weeks after Anticoagulation and acute event resolved and then repeat for confirmation in 12 weeks
-
Factor V Leiden or APC Resistance (G1681A)
- Initial: Clotting based assay
- Confirmatory: Factor V Leiden
- Factor II Prothrombin Mutation Analysis (G20210A)
-
Fasting Total Plasma Homocysteine Level
- Controversial as whether to check as the significance is questionable
- If high then check C677T as follows:
- Methylenetetrahydrofolate reductase (C677T)
VII. Labs: Evaluation of less common causes ($900)
-
General
- Indicated for strongly thrombophilic patients
- Testing includes all labs above
- Obtain lab testing 2 weeks after off Anticoagulants
- These conditions are highly thrombophilic
- Protein C Levels and Protein S Levels
- Antigenic (total protein) and functional levels
- Antithrombin III Level (HeparinCofactor assay)
- Plasma Factor VIII Level (increased)
VIII. Management
- High Risk Indications for life-long Anticoagulation
- Two or more spontaneous thrombotic events
- One spontaneous life-threatening event
- One spontaneous event with high risk cause
- Antiphospholipid Syndrome
- Antithrombin III deficiency
- More than one inherited abnormality
- Moderate Risk Indications for event-based prophylaxis
- One event with known provocative stimulus
IX. Resources
- University of Illinois Carle Cancer Center Resources
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| Definition (NCI) | A condition characterized by an abnormally high level of thrombi. Causes include thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, bone marrow disorders, and antiphospholipid antibody syndrome. |
| Definition (MSH) | A disorder of HEMOSTASIS in which there is a tendency for the occurrence of THROMBOSIS. |
| Concepts | Disease or Syndrome (T047) |
| MSH | D019851 |
| ICD10 | D68.59 |
| SnomedCT | 191302007, 76612001, 234467004 |
| English | Hypercoagulability, Hypercoagulabilities, Thrombophilias, thrombophilia (diagnosis), thrombophilia, Excessive Blood Clotting, Hypercoagulable state NOS, Thrombophilia NOS, Thrombophilia [Disease/Finding], thrombophilias, hypercoagulable state, hypercoagulability states, hypercoagulability, hypercoagulable states, Thrombophilia, Hypercoagulability State, Hypercoagulability state, Hypercoagulable state, Hypercoagulability state (finding), Thrombophilia (disorder), Hypercoagulability state, NOS |
| Dutch | trombofilie, Hypercoagulabiliteit, Trombofilie |
| Japanese | 栓友病, ｾﾝﾕｳﾋﾞｮｳ, 血栓形成傾向, 血栓傾向 |
| Swedish | Trombofili |
| Czech | trombofilie, Trombofilie |
| Finnish | Trombofilia |
| Russian | TROMBOFILIIA, ТРОМБОФИЛИЯ |
| Italian | Ipercoagulabilità, Trombofilia |
| Polish | Trombofilia, Nadkrzepliwość |
| Hungarian | Thrombophilia |
| Croatian | TROMBOFILIJA |
| Norwegian | Hyperkoagulasjon, Trombofili, Trombosetendens |
| Spanish | estado de hipercoagulabilidad (hallazgo), estado de hipercoagulabilidad, estado hipercoagulable, hipercoagulabilidad, trombofilia (trastorno), trombofilia, Trombofilia, Hipercoagulabilidad |
| French | Thrombophilie, Hypercoagulabilité |
| German | Thrombophilie, Hyperkoagulabilität |
| Portuguese | Hipercoagulabilidade, Trombofilia |
