ICD-10: D68.51
Activated protein C resistance
Clinical Information
Inclusion Terms
- Factor V Leiden mutation
Additional Information
Description
Activated Protein C Resistance (APCR) is a condition that significantly impacts the body's ability to regulate blood clotting. It is primarily associated with an increased risk of thrombosis, particularly venous thromboembolism. Below is a detailed clinical description and relevant information regarding the ICD-10 code D68.51, which specifically designates Activated Protein C Resistance.
Clinical Description of Activated Protein C Resistance
Definition
Activated Protein C Resistance is a disorder characterized by a reduced response to activated protein C (APC), a protein that plays a crucial role in the regulation of blood coagulation. This resistance can lead to an increased tendency for blood clot formation, which can result in serious complications such as deep vein thrombosis (DVT) and pulmonary embolism (PE) [2].
Pathophysiology
The most common cause of Activated Protein C Resistance is a genetic mutation in the Factor V gene, known as Factor V Leiden. This mutation leads to a variant of Factor V that is resistant to inactivation by activated protein C. As a result, the coagulation cascade remains activated longer than normal, increasing the risk of thrombotic events [3].
Clinical Presentation
Patients with Activated Protein C Resistance may present with:
- Thrombotic Events: The most common clinical manifestation is the occurrence of venous thromboembolism, which can happen at a young age or with minimal risk factors.
- Family History: A significant family history of thrombosis may be noted, as the condition is often inherited in an autosomal dominant pattern [2][3].
Diagnosis
Diagnosis of Activated Protein C Resistance typically involves:
- Coagulation Studies: These tests assess the response of plasma to activated protein C. A prolonged activated partial thromboplastin time (aPTT) may indicate resistance.
- Genetic Testing: Testing for the Factor V Leiden mutation can confirm the diagnosis in individuals suspected of having APCR [3].
Management
Management strategies for individuals diagnosed with Activated Protein C Resistance include:
- Anticoagulation Therapy: Patients may be prescribed anticoagulants, especially if they have experienced thrombotic events.
- Lifestyle Modifications: Recommendations may include avoiding prolonged immobility, maintaining a healthy weight, and managing other risk factors for thrombosis [2].
ICD-10 Code D68.51
The ICD-10 code D68.51 specifically refers to Activated Protein C Resistance. This code falls under the category of "Other specified coagulation defects" and is used for billing and coding purposes in healthcare settings. Accurate coding is essential for proper documentation, treatment planning, and insurance reimbursement.
Importance of Accurate Coding
Using the correct ICD-10 code is crucial for:
- Clinical Documentation: Ensures that the patient's medical records accurately reflect their condition.
- Insurance Claims: Facilitates appropriate reimbursement for services rendered related to the management of the condition.
- Epidemiological Tracking: Helps in the collection of data for public health monitoring and research on coagulation disorders [1].
Conclusion
Activated Protein C Resistance is a significant coagulation disorder that can lead to serious thrombotic complications. Understanding its clinical implications, diagnostic criteria, and management strategies is essential for healthcare providers. The ICD-10 code D68.51 serves as a critical tool for accurately documenting and managing this condition within the healthcare system. For patients with a family history of thrombosis or those presenting with unexplained thrombotic events, testing for APCR should be considered as part of a comprehensive evaluation.
Clinical Information
Activated Protein C (APC) resistance is a condition that significantly impacts the coagulation system, leading to an increased risk of thrombosis. The ICD-10-CM code for this condition is D68.51. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for effective diagnosis and management.
Clinical Presentation
Overview
Activated Protein C resistance is primarily associated with a genetic mutation, most commonly the Factor V Leiden mutation, which impairs the normal anticoagulant function of activated protein C. This condition can lead to venous thromboembolism (VTE), including deep vein thrombosis (DVT) and pulmonary embolism (PE) [12][14].
Signs and Symptoms
The clinical manifestations of APC resistance can vary widely among individuals, but common signs and symptoms include:
- Thrombotic Events: Patients may experience recurrent venous thromboembolism, which can manifest as:
- Deep Vein Thrombosis (DVT): Swelling, pain, and tenderness in the affected limb, often accompanied by warmth and redness.
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Pulmonary Embolism (PE): Symptoms may include sudden shortness of breath, chest pain (which may worsen with deep breathing), rapid heart rate, and coughing up blood.
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Family History of Thrombosis: Many patients with APC resistance have a family history of thrombotic events, indicating a hereditary predisposition to clotting disorders [12][14].
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Age of Onset: Thrombotic events often occur at a younger age than typically expected for the general population, particularly in individuals with the Factor V Leiden mutation [12].
Patient Characteristics
Certain characteristics are commonly observed in patients diagnosed with APC resistance:
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Genetic Predisposition: The majority of cases are linked to the Factor V Leiden mutation, which is more prevalent in certain populations, particularly those of European descent [12][14].
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Gender: There is a slight female predominance in cases of APC resistance, particularly in relation to pregnancy and the use of oral contraceptives, which can further increase the risk of thrombosis [12].
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Associated Conditions: Patients may have other risk factors for thrombosis, such as obesity, smoking, prolonged immobility, or the presence of other thrombophilic conditions [12][14].
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Recurrent Thrombotic Events: Many patients experience multiple episodes of thrombosis, which can complicate their clinical management and necessitate long-term anticoagulation therapy [12][14].
Conclusion
Activated Protein C resistance, coded as D68.51 in the ICD-10-CM, is a significant risk factor for thrombotic events, particularly in individuals with the Factor V Leiden mutation. The clinical presentation often includes recurrent DVT and PE, with a notable family history of thrombosis. Understanding the signs, symptoms, and patient characteristics associated with this condition is essential for healthcare providers to implement appropriate diagnostic and therapeutic strategies. Early identification and management can significantly reduce the risk of serious complications associated with this disorder.
Approximate Synonyms
Activated Protein C Resistance (APCR) is a condition that can lead to an increased risk of thrombosis due to the body's inability to effectively regulate blood clotting. The ICD-10-CM code for Activated Protein C Resistance is D68.51. Below are alternative names and related terms associated with this condition.
Alternative Names for Activated Protein C Resistance
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Factor V Leiden Mutation: This is the most common genetic cause of Activated Protein C Resistance, where a mutation in the Factor V gene leads to resistance against the anticoagulant effects of activated protein C[1].
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APC Resistance: A shorthand term often used in clinical settings to refer to Activated Protein C Resistance[2].
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Activated Protein C Resistance Syndrome: This term emphasizes the syndrome aspect of the condition, highlighting its clinical implications and association with thrombosis[3].
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Thrombophilia Due to APC Resistance: This term is used to describe the increased tendency to form blood clots as a result of the resistance to activated protein C[4].
Related Terms
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Thrombophilia: A broader term that encompasses various conditions, including Activated Protein C Resistance, that increase the risk of thrombosis[5].
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Hypercoagulable State: This term refers to a condition where there is an increased tendency for blood to clot, which can include various causes, including genetic factors like APC resistance[6].
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Venous Thromboembolism (VTE): A condition that includes deep vein thrombosis (DVT) and pulmonary embolism (PE), which can be a consequence of Activated Protein C Resistance[7].
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Primary Thrombophilia: This term refers to inherited conditions that predispose individuals to thrombosis, including those caused by APC resistance[8].
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Secondary Thrombophilia: This refers to acquired conditions that can lead to increased clotting risk, which may coexist with genetic factors like APC resistance[9].
Conclusion
Understanding the alternative names and related terms for ICD-10 code D68.51 is crucial for healthcare professionals involved in diagnosing and managing conditions associated with thrombosis. Recognizing these terms can aid in effective communication and documentation in clinical practice. If you need further information on the implications of Activated Protein C Resistance or its management, feel free to ask!
Diagnostic Criteria
Activated Protein C Resistance (APCR) is a condition that can lead to an increased risk of thrombosis due to a deficiency in the body's ability to regulate blood clotting. The ICD-10-CM code D68.51 specifically refers to this condition. The diagnosis of APCR typically involves a combination of clinical evaluation and laboratory testing. Below are the key criteria and methods used for diagnosing this condition.
Clinical Criteria
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Patient History:
- A thorough medical history is essential, focusing on any previous thrombotic events (e.g., deep vein thrombosis, pulmonary embolism).
- Family history of thrombosis or known inherited thrombophilias can also be significant. -
Symptoms:
- Patients may present with symptoms related to thrombosis, such as swelling, pain, or redness in the limbs, or symptoms of pulmonary embolism (e.g., shortness of breath, chest pain).
Laboratory Testing
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Activated Protein C Resistance Test:
- This is the primary test used to diagnose APCR. It assesses the ability of activated protein C to inactivate factor Va in the presence of a patient's plasma.
- A reduced response indicates resistance, which is often due to the presence of Factor V Leiden mutation. -
Genetic Testing:
- Testing for the Factor V Leiden mutation is crucial, as this genetic mutation is the most common cause of APCR.
- Genetic testing can confirm the presence of the mutation, which is inherited in an autosomal dominant manner. -
Coagulation Profile:
- A complete coagulation profile may be performed to rule out other causes of thrombophilia. This includes tests such as prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet count. -
Other Thrombophilia Tests:
- Additional tests may include checking for deficiencies in natural anticoagulants (e.g., antithrombin, protein C, and protein S) and the presence of antiphospholipid antibodies.
Diagnostic Criteria Summary
- Positive Activated Protein C Resistance Test: Indicates resistance to activated protein C.
- Presence of Factor V Leiden Mutation: Confirmed through genetic testing.
- Clinical History of Thrombosis: Documented episodes of venous thromboembolism.
- Exclusion of Other Causes: Ensuring that other potential causes of thrombosis are ruled out through comprehensive testing.
Conclusion
The diagnosis of Activated Protein C Resistance (ICD-10 code D68.51) relies on a combination of clinical assessment and specific laboratory tests. The activated protein C resistance test, along with genetic testing for Factor V Leiden, plays a pivotal role in confirming the diagnosis. Understanding these criteria is essential for healthcare providers to effectively identify and manage patients at risk for thrombotic events.
Treatment Guidelines
Activated Protein C Resistance (APCR), classified under ICD-10 code D68.51, is a condition that increases the risk of thrombosis due to a deficiency in the anticoagulant function of activated protein C. This condition is often associated with Factor V Leiden mutation, which is the most common inherited form of thrombophilia. The management of APCR primarily focuses on preventing thrombotic events and managing any existing thrombosis. Below, we explore standard treatment approaches for this condition.
Standard Treatment Approaches
1. Anticoagulation Therapy
Anticoagulation is the cornerstone of treatment for patients with activated protein C resistance, especially those with a history of venous thromboembolism (VTE). The following anticoagulants are commonly used:
- Warfarin: This vitamin K antagonist is often prescribed for long-term management. Patients typically require monitoring of their International Normalized Ratio (INR) to ensure therapeutic levels are maintained.
- Direct Oral Anticoagulants (DOACs): Medications such as rivaroxaban, apixaban, and dabigatran may be used as alternatives to warfarin. These agents do not require routine monitoring and have a more predictable pharmacokinetic profile, making them easier to manage in outpatient settings[1][2].
2. Management of Acute Thrombotic Events
In cases where a patient presents with an acute thrombotic event, immediate treatment is crucial:
- Thrombolysis: In severe cases, thrombolytic therapy may be indicated to dissolve clots quickly, particularly in life-threatening situations such as pulmonary embolism or extensive deep vein thrombosis (DVT).
- Unfractionated Heparin (UFH): This is often used in the hospital setting for rapid anticoagulation, especially in patients with acute VTE. It can be transitioned to warfarin or a DOAC once the patient is stable[3].
3. Long-term Management and Prevention
For patients with a history of thrombosis, long-term anticoagulation may be necessary:
- Duration of Therapy: The length of anticoagulation therapy depends on various factors, including the presence of risk factors, the type of thrombosis, and whether it was provoked or unprovoked. Generally, patients with APCR may require extended therapy, often for at least 3 to 6 months, and sometimes indefinitely if they have recurrent events[4].
- Lifestyle Modifications: Patients are advised to adopt lifestyle changes that can help reduce the risk of thrombosis, such as maintaining a healthy weight, engaging in regular physical activity, and avoiding prolonged immobility[5].
4. Genetic Counseling and Testing
Given the hereditary nature of APCR, genetic counseling may be beneficial for patients and their families. Testing for Factor V Leiden mutation can help identify at-risk family members and guide management strategies[6].
5. Monitoring and Follow-up
Regular follow-up appointments are essential to monitor the effectiveness of anticoagulation therapy and to adjust dosages as necessary. Patients should also be educated about the signs and symptoms of thrombosis to ensure prompt medical attention if needed[7].
Conclusion
The management of Activated Protein C Resistance involves a multifaceted approach centered on anticoagulation therapy, both for acute events and long-term prevention. Individualized treatment plans, regular monitoring, and lifestyle modifications play critical roles in reducing the risk of thrombotic complications. As research continues to evolve, staying informed about the latest guidelines and treatment options is essential for healthcare providers managing patients with this condition.
References
- Anticoagulation Management Tool User Manual.
- Pub 100-04 Medicare Claims Processing.
- Venous and Arterial Thrombosis Risk Testing.
- medical-policy-genetic-testing-for-inherited-thrombophilia.
- ICD-10 International statistical classification of diseases.
- 2025 ICD-10-CM Diagnosis Code D68.51.
- ICD-10-CM Diagnosis Code D68.51 - Activated protein C resistance.
Related Information
Description
- Blood clotting regulation disorder
- Increased risk of venous thromboembolism
- Deep vein thrombosis and pulmonary embolism complications
- Genetic mutation in Factor V gene
- Resistant to activated protein C inactivation
- Coagulation cascade remains active longer
- Risk of thrombotic events increased
Clinical Information
- Genetic mutation impairs activated protein C function
- Factor V Leiden mutation most common cause
- Venous thromboembolism risk increased significantly
- Deep vein thrombosis and pulmonary embolism common
- Recurrent thrombotic events frequent complication
- Family history of thrombosis often present
- Younger age of onset than general population
- Female predominance in cases with pregnancy or oral contraceptives
Approximate Synonyms
- Factor V Leiden Mutation
- APC Resistance
- Activated Protein C Resistance Syndrome
- Thrombophilia Due to APC Resistance
- Hypercoagulable State
- Venous Thromboembolism (VTE)
- Primary Thrombophilia
- Secondary Thrombophilia
Diagnostic Criteria
- Thorough medical history review
- Family history of thrombosis significant
- Patient symptoms consistent with thrombosis
- Reduced response on Activated Protein C Test
- Presence of Factor V Leiden mutation confirmed
- Clinical history of venous thromboembolism documented
- Other potential causes excluded through testing
Treatment Guidelines
- Anticoagulation therapy is primary treatment
- Warfarin and DOACs are common anticoagulants
- Monitor INR for warfarin patients
- Thrombolysis for severe acute thrombotic events
- UFH in hospital setting for rapid anticoagulation
- Long-term therapy for high-risk patients
- Lifestyle modifications to reduce risk
- Genetic counseling and testing recommended
Related Diseases
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