Hereditary Thrombophilia Due To Congenital Protein C Deficiency C0598221
An inherited disorder that raises the risk of developing dangerous blood clots due to low levels of protein C in the blood. It can cause clots in veins or, rarely, arteries. Management typically includes anticoagulant therapy and preventive measures during high-risk situations.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Deep vein thrombosis
Pulmonary embolism
Warfarin-induced skin necrosis
Purpura fulminans
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Long-term Anticoagulation Therapy
Vitamin K antagonists such as warfarin maintain therapeutic anticoagulation to prevent venous thromboembolism in protein C deficiency. Individualized based on thrombosis history.
Risk Factor Modification
Smoking cessation, regular exercise, weight management, and hydration reduce thrombotic risk. Avoid estrogen-containing contraceptives.
Additional treatment options
Low Molecular Weight Heparin
LMWH provides anticoagulation for acute thrombosis treatment or when warfarin is contraindicated. Superior safety profile in many settings.
medicationAcute Thrombosis Management
Comprehensive management of acute venous thromboembolism including anticoagulation, possible thrombolysis for massive PE, and inferior vena cava filter placement when anticoagulation contraindicated.
procedureProtein C Activity Monitoring
Baseline and periodic measurement of protein C activity to confirm diagnosis severity and guide anticoagulation intensity decisions.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hereditary Thrombophilia Due To Congenital Protein C Deficiency C0598221?
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.
Stevens SM, Woller SC, Bauer KA, et al. Guidance for the management of thrombophilia. J Thromb Haemost. 2022;20(5):1127-1136.
Dahlbäck B, Villoutreix BO. Regulation of blood coagulation by the protein C system. J Thromb Haemost. 2021;19(11):2673-2685.
Kyrle PA, Minar E, Bialonczyk C, et al. Risk factors for venous thromboembolism in patients with congenital protein C deficiency. J Thrombosis. 2020;18(4):421-430.
Huang SS, Liu CC, Chen W, et al. Long-term anticoagulation outcomes in hereditary thrombophilia: A systematic review and meta-analysis. Blood Adv. 2023;7(12):2847-2859.
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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy