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Factor IX Activity
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No Fasting Required
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Coagulation factor activity measurement.
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Factor IX Activity Test Guide
- Why is it done?
- Measures the functional activity level of Factor IX (Christmas factor), a crucial blood clotting protein involved in the intrinsic coagulation pathway
- Diagnoses Factor IX deficiency (hemophilia B or Christmas disease) in patients with prolonged bleeding or family history of bleeding disorders
- Monitors anticoagulant therapy effectiveness, particularly warfarin treatment which affects vitamin K-dependent factors including Factor IX
- Evaluates unexplained prolonged prothrombin time (PT) or activated partial thromboplastin time (aPTT)
- Assesses bleeding risk prior to surgical procedures or dental work in at-risk patients
- Guides dosing decisions for factor replacement therapy in hemophilia B patients
- Normal Range
- Normal Range: 75-125% (or 0.75-1.25 IU/mL)
- Units of Measurement: Percent (%) of normal activity or International Units per milliliter (IU/mL)
- Interpretation of Results:
- Normal (75-125%): Adequate Factor IX activity; normal clotting function for this factor
- Low (<75%): Factor IX deficiency; increased bleeding risk; may indicate hemophilia B or vitamin K deficiency
- Critically Low (<10%): Severe deficiency with significant spontaneous bleeding risk; requires urgent intervention
- High (>125%): Elevated Factor IX activity; potential thrombotic risk; may occur with malignancy or certain inflammatory conditions
- Reference Ranges: May vary slightly between laboratories depending on methodology; always compare results to the specific laboratory's reference range provided on the test report
- Interpretation
- Mild Deficiency (50-74%): May have minimal symptoms; bleeding risk increases with trauma or surgical procedures; prophylactic therapy often not required unless planning invasive procedures
- Moderate Deficiency (10-50%): Typical hemophilia B presentation; spontaneous bleeding may occur; requires factor replacement for bleeding episodes and before invasive procedures
- Severe Deficiency (<10%): Spontaneous bleeding common (hemarthroses, muscle hematomas, GI bleeding); requires regular prophylactic factor replacement therapy to prevent bleeding complications
- Factors Affecting Results:
- Vitamin K deficiency decreases Factor IX levels (also affects Factors II, VII, X)
- Warfarin and other anticoagulants reduce Factor IX production
- Liver disease impairs synthesis of vitamin K-dependent factors
- Circulating inhibitors or antibodies against Factor IX (alloimmunization) interfere with activity measurement
- Recent factor replacement therapy or transfusion may temporarily elevate results
- Specimen collection techniques (improper mixing with anticoagulant, hemolysis) may affect accuracy
- Clinical Significance of Result Patterns:
- Isolated low Factor IX with normal PT suggests factor-specific deficiency or hemophilia B
- Low Factor IX with low other vitamin K-dependent factors suggests vitamin K deficiency or warfarin effect
- Disproportionately low Factor IX compared to Factor II and X may indicate specific inhibitor
- Associated Organs
- Primary Organ Systems:
- Hematopoietic/Hematologic System: Factor IX is synthesized in the liver and involved in blood coagulation cascade
- Hepatic System: Liver produces Factor IX; liver disease directly impairs factor synthesis
- Medical Conditions Associated with Abnormal Results:
- Hemophilia B (Factor IX Deficiency): X-linked recessive inherited bleeding disorder; presents with easy bruising, prolonged bleeding, spontaneous hemarthroses
- Vitamin K Deficiency: Results from malabsorption, antibiotic use, or dietary insufficiency; affects all vitamin K-dependent factors (II, VII, IX, X)
- Liver Disease: Cirrhosis, hepatitis, fulminant liver failure impair hepatic synthesis of coagulation factors; low Factor IX often indicates advanced liver disease
- Disseminated Intravascular Coagulation (DIC): Widespread coagulation activation consumes Factor IX and other clotting factors; associated with sepsis, trauma, malignancy
- Nephrotic Syndrome: Protein wasting leads to loss of clotting factors in urine
- Malignancy: Can increase Factor IX (acute phase response) or decrease it (consumption in thrombosis, liver metastases)
- Alloimmunization (Factor IX Inhibitor): Development of antibodies against Factor IX in hemophilia B patients receiving replacement therapy; complicates treatment significantly
- Potential Complications with Abnormal Results:
- Excessive Bleeding: Spontaneous or post-traumatic hemorrhage with low Factor IX levels; may lead to anemia and hemodynamic compromise
- Hemarthrosis and Muscle Hematomas: Repeated bleeding into joints and muscles causing chronic pain, disability, and arthropathy in severe hemophilia
- Intracranial Hemorrhage: Life-threatening complication in severe deficiency; can result from minor trauma
- Thrombosis Risk: Elevated Factor IX levels increase risk of thromboembolism and stroke
- Primary Organ Systems:
- Follow-up Tests
- Recommended if Results are Abnormal:
- Other Vitamin K-Dependent Factors (II, VII, X): Determine if deficiency is isolated to Factor IX or affects multiple vitamin K-dependent factors, pointing to vitamin K deficiency or liver disease
- Factor IX Inhibitor Assay (Bethesda Assay): Detects inhibitory antibodies in patients with hemophilia B; essential before prescribing factor replacement
- Prothrombin Time (PT): Assesses vitamin K-dependent factor pathway; helps clarify if deficiency is specific or multifactorial
- Activated Partial Thromboplastin Time (aPTT): Detects intrinsic and common pathway defects; prolonged aPTT with low Factor IX typical for hemophilia B
- Liver Function Tests (LFTs): Evaluate hepatic function if low Factor IX suspected from liver disease
- Bleeding Time or PFA-100: Assess platelet function if abnormal bleeding suspected despite normal factor levels
- Further Investigations:
- Genetic testing for hemophilia B mutations in confirmed Factor IX deficiency; especially important for family counseling and inheritance pattern determination
- Thrombin Generation Assay: Advanced coagulation test providing global assessment of hemostatic potential; useful for predicting bleeding risk
- Imaging studies (ultrasound, MRI) if internal bleeding suspected based on symptoms
- Monitoring Frequency:
- Hemophilia B Patients: Baseline Factor IX activity level established at diagnosis; monitored periodically (annually or as clinically indicated) to assess factor stability and detect development of inhibitors
- On Factor Replacement Therapy: Factor IX levels measured at regular intervals to ensure therapeutic goals are met; more frequent testing if switching factor products or adjusting dosing
- Patients on Warfarin: Factor IX monitored periodically during warfarin therapy; more frequent testing when initiating, adjusting dose, or if bleeding complications develop
- Patients with Liver Disease: Factor IX activity may be monitored as marker of hepatic synthetic function; deteriorating levels indicate worsening liver disease
- Related Complementary Tests:
- Complete Blood Count (CBC): Detects anemia from chronic bleeding; monitors for thrombocytopenia
- D-dimer: Screens for thrombotic complications in patients with elevated Factor IX
- Fibrinogen Level: Assesses common pathway of coagulation cascade
- Recommended if Results are Abnormal:
- Fasting Required?
- Fasting: NO - Fasting is not required for Factor IX Activity testing
- Special Patient Preparation:
- Patient may eat and drink normally before blood draw
- Schedule blood draw at consistent time of day if possible, as coagulation factors may show diurnal variation
- Avoid arm trauma, venipuncture from sites with hematomas, or phlebotomy from upper extremity with known hemarthrosis
- Patients on factor replacement therapy should be tested at consistent intervals relative to dosing (usually trough levels before next infusion, or peak levels 15-30 minutes after infusion)
- Medications to Avoid:
- No specific medications need to be discontinued for test accuracy; however, inform laboratory of all medications being taken
- Note timing if on warfarin or other anticoagulants, as these medications affect Factor IX results (intentional for monitoring purposes)
- Aspirin and NSAIDs may increase bleeding risk in patients with deficiency but do not affect test results directly
- Specimen Collection Requirements:
- Blood collected in sodium citrate tube (blue-top) - exact ratio of blood to anticoagulant is critical for accurate results
- Tube must be filled to the proper mark to maintain 1:9 blood to anticoagulant ratio
- Gentle mixing by inversion immediately after collection; do not shake vigorously as this causes hemolysis
- Specimen should be transported to laboratory promptly and kept at room temperature (do not refrigerate) as chilling may cause platelet activation
- Avoid bloody or hemolyzed specimens; if collection is difficult, discard first tube and recollect to ensure accuracy
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