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Factor IX Activity

Blood
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Report in 72Hrs

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At Home

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No Fasting Required

Details

Coagulation factor activity measurement.

2,0722,960

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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
  • 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
  • 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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