ICD-10-CM Coding for Hypercoagulable States

Hypercoagulable states are blood clotting disorders that increase the risk of deep vein thrombosis or embolic disease. This is due to the presence of either an inherited or acquired clotting factor. About 80% of patients with blood clots have been found to have either an inherited or acquired clotting disorder. These blood clots can be lethal, and some require life-long therapy. Hypercoagulable state is also known as thrombophilia.  

Inherited Clotting Disorders

• Factor V Leiden mutation—this is the most common inherited factor associated hypercoagulopathy 
• Prothrombin G20210A mutation—this is the second most common inherited factor associated hypercoagulopathy 
• Protein C and Protein S deficiency—this is uncommon, but these deficiencies fail to regulate the coagulation process properly 
• Antithrombin (or antithrombin III) deficiency—this is a very rare autosomal dominant disorder 
• Other—there are other rare inherited disorders that affect coagulopathy including dysfibrinogenemia, plasminogen deficiency, heparin cofactor II deficiency, factor XII deficiency, and elevated clotting factor levels 

Acquired Causes of Hypercoagulopathy 

• Malignancy—malignancy may be documented as the cause of thrombosis. The type of malignancy may be known or occult (unknown) 
• Medications—including heparin, oral contraceptives, other hormone replacement therapy, tamoxifen and multiple other medications 
• Pregnancy—increases the risk of thrombosis due to hormonal and physical changes to the body. The enlarged uterus is also felt to compress the pelvic blood vessels which increases the likelihood of the creation of clots 
• Trauma/Surgery—increases the risk of thrombosis due to decreased venous blood flow, immobilization, increased levels of tissue factor, and alterations in the balance of the endogenous procoagulants and anticoagulants. Clots occur most commonly after orthopedic, vascular, and neurosurgical injuries/surgeries 
• Chronic kidney disease/Nephrotic syndrome—thought to be due to having too much protein in the urine 
• Hyperhomocysteinemia (both acquired and inherited) Heparin induced thrombocytopenia and thrombus (HTT) 
• Myeloproliferative Disorders/Malignancy—polycythemia vera, essential thrombocythemia, and primary myelofibrosis 
• Antiphospholipid Antibody Syndrome—presence of antiphospholipid antibodies/ thrombocytopenia 
• Immobilization—due to physical conditions such as obesity, recovery from trauma or surgery, disease that inhibits patient mobility, or lengthy periods of sitting while traveling.  
• Other—such as rheumatologic disease, inflammatory disease, smoking, lupus, autoimmune diseases, HIV/AIDS, hemoglobinuria (paroxysmal nocturnal hemoglobinuria [PNH]) among others

Some of the disorders above (either inherited or acquired) are rare but there are some that are considered the most common. These can be remembered by using this mnemonic “DAMN THROMBUS.” 

Deficiencies in coagulation (or alterations). These include the disorders such as protein C and S, heparin cofactor, antithrombin III, fibrinogen, factor XIII, prothrombin, and plasminogen
Antiphospholipid antibody syndrome
Malignancy
Nephrotic syndrome/CKD
Traumatic
Hyperhomocysteinemia or hemoglobinuria
Rheumatologic disease
Oral contraception or other medications
Myeloproliferative disorders
Being pregnant
Unknown
Surgical

Anticoagulation is the most common treatment for thrombus developing due to any of these disorders. The type and duration of anticoagulation will depend on the site of the thrombus and whether the condition is recurrent. With recurrence, or when there is a chance of active bleeding in the patient, inferior vena cava filter (IVC) may be placed. If the patient has profound thrombosis, thrombolytic therapy may also be indicated to restore blood flow more quickly.  

Additional Facts About Hypercoagulopathy 

• Primary hypercoagulable state = inherited clotting disorders 
  • The most common cause of inherited hypercoagulable state is activated protein C resistance (factor V Leiden) 
  • Protein C and S deficiencies are vitamin K dependent 
• Secondary hypercoagulable state = primarily acquired clotting disorders 
  • The most common cause of acquired hypercoagulable state is antiphospholipid antibody syndrome 
  • Malignancy/cancer is the second most common cause of hypercoagulable state and accounts for 10% to 20% of spontaneous venous thromboses 
    • 90% of patients that have cancer will develop a clotting abnormality 
• The cause of the hypercoagulopathy determines the type and duration of treatment 
• The cause of the hypercoagulable state can be correctly diagnosed in 80% to 90% of patients 
• Testing for specific causes of hypercoagulability can be very costly 
• To avoid hypercoagulopathy, remove or avoid risk factors that contribute to a hypercoagulable state (smoking, oral birth control, prolonged immobilization, etc.) 
• Even inherited hypercoagulability disorders can be influenced by environmental factors and habits

 ICD-10-CM Coding Considerations 

When assigning codes for documentation of hypercoagulopathy, the coder should keep the following in mind.  

• If a diagnosis of hypercoagulable state is documented, is it coded? That all depends on if it meets the ICD-10-CM reporting criteria. Chances are, the patient is under treatment or monitoring for the condition, for which follow up is normally required. This would indicate a code should be assigned. If the documentation is unclear, a query would be needed to clarify if this diagnosis was clinically significant on the current admission.  
• The documentation states that the patient has a venous thrombus. Does this support coding of the thrombus and a hypercoagulable state? NO! The presence of a thrombus does not necessarily mean that the patient has a hypercoagulable state. The physician must document a diagnosis of hypercoagulopathy before it can be coded.  
• A prolonged prothrombin time on lab results is not the same as hypercoagulopathy. This is reported with R79.1 Abnormal coagulation profile 
• Bleeding due to anticoagulation therapy (administered correctly) is coded as an adverse effect and NOT a complication of treatment. 
• For bleeding such as hemoptysis, hematuria, hematemesis, hematochezia, etc., that is associated with a drug, as part of anticoagulation therapy, assign code D68.32, Hemorrhagic disorder due to extrinsic circulating anticoagulants 
• If the documentation says only “hypercoagulable state”, can the coder review the patient’s coexisting diseases/diagnoses and decide whether the patient’s hypercoagulopathy is primary or secondary? NO! Only the physician can make the link between the cause/effect. However, a query could be initiated for clarification. 

 Take Aways 

• Hypercoagulable states are blood clotting disorders that increase the risk of deep vein thrombosis or embolic disease. This is due to the presence of either an inherited or acquired clotting factor.  
• Hypercoagulable state is also known as thrombophilia.  
• Primary hypercoagulable state = inherited clotting disorders 
• Secondary hypercoagulable state = primarily acquired clotting disorders 
• The cause of the hypercoagulopathy determines the type and duration of treatment, but anticoagulation is the most common treatment. 
• Not all patients with a thrombosis have a hypercoagulable state or disorder 
• Definitive coding should not be made from abnormal lab results related to bleeding time. 
• Correct coding of hypercoagulopathy requires documentation that clearly outlines the connections between hypercoagulable states and their causes.

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