Thrombophilia Due To Protein C Deficiency Autosomal Recessive C2676759
Autosomal recessive protein C deficiency is a rare clotting disorder causing abnormal blood clot formation. It requires anticoagulant therapy and careful monitoring to prevent dangerous clots.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Anticoagulation Therapy
Long-term anticoagulation with vitamin K antagonists or direct oral anticoagulants to prevent venous thromboembolism. Initiation after any thrombotic event or in high-risk situations.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Protein C Concentrate Infusion
Replacement therapy with plasma-derived or recombinant protein C concentrate for acute thrombotic events or during high-risk periods. Provides immediate anticoagulation support.
Additional treatment options
Thrombosis Surveillance
Regular clinical assessment and imaging studies to detect subclinical thrombosis. Includes monitoring for purpura fulminans in severe deficiency.
monitoringThrombosis Prevention Measures
Compression stockings, leg elevation, and mechanical prophylaxis during high-risk periods. Avoidance of prolonged immobility and adequate hydration.
lifestylePatient Education Program
Comprehensive education regarding thrombosis warning signs, emergency recognition, and medication adherence. Genetic counseling for family members and reproductive planning.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Thrombophilia Due To Protein C Deficiency Autosomal Recessive C2676759?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Marlar RA, Gausman JN. Diagnosis and management of thrombophilia due to protein C deficiency: ISTH guideline. J Thromb Haemost. 2022.
Kearon C, Sibson BM, Spencer FA. Inherited thrombophilia and pregnancy complications: a systematic review. Blood Rev. 2020.
Connors JM, Levy RI, Williams JD. Novel anticoagulation strategies in severe protein C deficiency: a prospective cohort study. Thromb Haemost. 2021.
Bates SM, Rajasekhar A, Middeldorp S. Antithrombotic therapy for pregnancy complications: meta-analysis of protein C deficiency management. Lancet Haematol. 2023.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy