Short answer · Medically reviewed summary · Last updated: 2026-04-07

Antithrombin III deficiency is classified under ICD-10 code D68.51 (Congenital antithrombin III deficiency) and ICD-9 code 289.81 (Primary hypercoagulable state). These medical classification codes are essential for healthcare providers to document this rare, hereditary blood disorder characterized by an increased risk of venous thromboembolism. What is the clinical significance of Antithrombin III deficiency? Antithrombin III deficiency is a rare, inherited thrombophilia that impairs the body's natural ability to prevent blood clots.

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ICD10 code of Antithrombin III deficiency and ICD9 code

ICD-10 and ICD-9 codes for Antithrombin III deficiency, with classification details for clinicians, coders and patients.

ICD9 and ICD10 codes of Antithrombin III deficiency

Antithrombin III deficiency is classified under ICD-10 code D68.51 (Congenital antithrombin III deficiency) and ICD-9 code 289.81 (Primary hypercoagulable state). These medical classification codes are essential for healthcare providers to document this rare, hereditary blood disorder characterized by an increased risk of venous thromboembolism.



What is the clinical significance of Antithrombin III deficiency?


Antithrombin III deficiency is a rare, inherited thrombophilia that impairs the body's natural ability to prevent blood clots. Antithrombin is a protein that serves as a vital anticoagulant; when levels are low or function is impaired, patients are at a significantly higher risk for deep vein thrombosis (DVT) and pulmonary embolism (PE). Because Antithrombin III deficiency is a genetic condition, it is often identified only after a patient experiences their first unexplained clot or through family screening following an affected relative's diagnosis.



How is Antithrombin III deficiency diagnosed and coded?


Diagnosis typically involves specialized blood tests that measure both the antigenic level and the functional activity of the protein. When a physician confirms Antithrombin III deficiency, they use specific diagnostic coding to ensure appropriate insurance coverage and clinical management. While ICD-10 code D68.51 is standard for congenital forms, clinicians must also distinguish between Type I (quantitative deficiency) and Type II (qualitative/functional defect) to provide accurate treatment plans.



Is Antithrombin III deficiency hereditary?


Yes, Antithrombin III deficiency follows an autosomal dominant pattern of inheritance. This means that an individual only needs to inherit the mutated gene from one parent to be at risk for the condition. Genetic counseling is highly recommended for families, as the prevalence of symptomatic Antithrombin III deficiency in the general population is estimated to be between 1 in 500 and 1 in 5,000. Understanding these hereditary patterns helps family members make informed decisions regarding prophylactic anticoagulation, especially before high-risk events like surgery or pregnancy.



What are the common management strategies?


Managing this condition requires a multidisciplinary approach, often involving hematologists and vascular specialists. Based on the experiences of the 42 members within the DiseaseMaps community, the following management strategies are commonly discussed:



  • Long-term or prophylactic anticoagulation therapy (e.g., heparin or warfarin).

  • Antithrombin concentrate replacement therapy during high-risk scenarios such as surgery, trauma, or childbirth.

  • Strict avoidance of oral contraceptives or hormone replacement therapy, which can exacerbate clotting risks.

  • Regular monitoring of coagulation markers by a specialized hematology team.

  • Lifestyle modifications focused on reducing venous stasis, such as compression stockings and regular movement during long travel.



Next steps



  • Schedule an appointment with a hematologist specializing in inherited thrombophilias to establish a baseline management plan.

  • Consult with a genetic counselor to discuss family screening options for siblings and children.

  • Join the DiseaseMaps.org community to connect with other patients who share their experiences living with Antithrombin III deficiency.

  • Ensure your medical records are updated with the correct ICD-10 code (D68.51) to facilitate appropriate care coordination during emergencies.



Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.



References



  • National Institutes of Health (NIH) Genetic and Rare Diseases (GARD) Information Center: Antithrombin deficiency.

  • Orphanet: Antithrombin III deficiency (ORPHA:93922).

  • Online Mendelian Inheritance in Man (OMIM): Antithrombin III deficiency (Entry #613118).

  • National Heart, Lung, and Blood Institute (NHLBI): Thrombophilia overview.

Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2026-04-07
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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