Hematology and Oncology Book

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HypercoagulableAka: Hypercoagulability, Thrombophilia

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  1. History suggestive of Hypercoagulable State
    1. Thrombosis at a young age (age under 50 years)
    2. Family History of thrombosis
    3. Recurrent Thrombosis
    4. Thrombosis in an unusual site
    5. Pregnancies complicated by frequent Miscarriage
  2. Etiologies
    1. Primary hypercoagulable States (Hereditary)
      1. Common Causes
        1. Factor V Leiden Defect
        2. Prothrombin 20210
        3. Homocystinuria or Hyperhomocysteinemia
      2. Uncommon Causes
        1. Antithrombin III deficiency
        2. Protein C Deficiency
        3. Protein S Deficiency
        4. Factor VIII Increased
        5. Fibrinolysis
        6. Dysfibrinogenemia
    2. Secondary or Acquired Hypercoagulable States
      1. Pregnancy
      2. Estrogen sources
        1. Oral Contraceptives
        2. Estrogen Replacement Therapy
        3. Tamoxifen
      3. Surgery
      4. Trauma
      5. Infection or Sepsis
      6. Malignancy
        1. Cancer in idiopathic hypercoagulability: 20%
      7. Myeloproliferative disorder
      8. Hyperlipidemia
      9. Homocystinuria
      10. Lupus Inhibitor
      11. Antiphospholipid Antibodies
      12. Nephrotic Syndrome
  3. Labs
    1. General
      1. Hold blood for tests below before Anticoagulation
    2. Risk of thrombophilia determines level of testing
      1. Unlikely to be thrombophilic
        1. Criteria
          1. First episode of Venous Thromboembolism and
          2. Known Thromboembolism Risk Factors and
          3. No Family History of thromboembolism
            1. No first degree relative under age 50 years
        2. Testing
          1. No testing indicated
      2. Weakly thrombophilic
        1. Criteria
          1. Age over 50 years and
          2. First thromboembolic episode and
            1. No known Thromboembolism Risk Factors
          3. No Family History of thromboembolism
            1. No first degree relative under age 50 years
        2. Testing
          1. Evaluation of common causes (see below)
      3. Strongly thrombophilic
        1. Criteria
          1. Age <50, no known Thromboembolism Risk Factors or
          2. Recurrent thromboembolic episode or
          3. Family History of thromboembolism
            1. First degree relative under age 50 years
        2. Testing
          1. Evaluation of common causes and
          2. Evaluation of less common causes (see below)
    3. Evaluation of common causes ($250)
      1. Complete Blood Count with platelets and morphology
      2. Prothrombin Time
      3. Partial Thromboplastin Time
      4. Connective Tissue Disorder tests
        1. Antinuclear Antibody Test (ANA)
        2. Antiphospholipid Antibody tests
          1. Clotting Assay for Lupus Anticoagulant
          2. ELISA for Anticardiolipin Antibodies
      5. Factor V Leiden or APC Resistance (G1681A)
        1. Initial: Clotting based assay
        2. Confirmatory: Factor 5 Leiden
      6. Fasting Total Plasma Homocysteine Level
        1. If high then check C677T as follows:
        2. Methylenetetrahydrofolate reductase (C677T)
      7. Factor II Prothrombin Mutation Analysis (G20210A)
    4. Evaluation of less common causes ($900)
      1. General
        1. Indicated for strongly thrombophilic patients
        2. Testing includes all labs above
        3. Obtain lab testing 2 weeks after off Warfarin
      2. Protein C Levels and Protein S Levels
        1. Antigenic (total protein) and functional levels
      3. Antithrombin III Level (Heparin cofactor assay)
      4. Plasma Factor VIII Level (increased)
  4. Management
    1. High Risk Indications for life-long Anticoagulation
      1. Two or more spontaneous thrombotic events
      2. One spontaneous life-threatening event
      3. One spontaneous event with high risk cause
        1. Antiphospholipid Syndrome
        2. Antithrombin III deficiency
        3. More than one inherited abnormality
    2. Moderate Risk Indications for event-based prophylaxis
      1. One event with known provocative stimulus
  5. Resources
    1. University of Illinois Carle Cancer Center Resources
      1. http://www.med.uiuc.edu/hematology/index.htm
  6. References
    1. Bauer (2001) Ann Intern Med 135:367
    2. Harris (1997) Am Fam Physician 56(6):1591
    3. Kyrle (2000) N Engl J Med 343:457
    4. Nachman (1996) Ann Intern Med 119:819
    5. Thomas (1997) Ann Intern Med 126:638

Thrombophilia (C0398623)

Definition (MSH)A disorder of HEMOSTASIS in which there is a tendency for the occurrence of THROMBOSIS.
ConceptsDisease or Syndrome (T047)
MSHD019851
EnglishHypercoagulabilities, Hypercoagulability, Hypercoagulability state, Hypercoagulable state, Thrombophilia, Thrombophilias
Spanishestado de hipercoagulabilidad, estado hipercoagulable, hipercoagulabilidad, trombofilia
Parent ConceptsHematological Disease (C0018939), Blood Coagulation Disorders (C0005779), Hemostatic system finding (C1292125), Duplicate concept (C1274013)
SourcesCOSTAR, MSH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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