Hypercoagulable States

Hypercoagulable states are blood disorders that increase the risk of deep vein thrombosis or embolic disease. The state is either inherited or acquired. 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. The patients that fall into one of these disorders have an increased tendency to develop blood clots. This is due to the presence of either an inherited factor or an acquired factor.

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 include dysfibrinogenemia, plasminogen deficiency, heparin cofactor II deficiency, factor XII deficiency, and elevated clotting factor levels

Acquired Clotting Disorders

  • Malignancy—coders see this often documented as the cause of thrombosis. These malignancies are either known or occult
  • Medications—including heparin, oral contraceptives, other hormone replacement therapy, tamoxifen and multiple other additional medications
  • Pregnancy—increases the risk of thrombosis due to hormonal and physical changes to the body. The enlarged uterus is felt to also compress the pelvic blood vessels
  • 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 most common 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 condition such as obesity, recovering from trauma or surgery, disease that prevents a patient from being mobile, or lengthy airplane travel, which is also known as “economy class syndrome”
  • Other—such as rheumatologic disease, inflammatory diseases, smoking, lupus, autoimmune diseases, HIV/AIDS, hemoglobinuria (paroxysmal nocturnal hemoglobinuria [PNH]) just to list a few

Some of the disorders above (either inherited or acquired) are rare but there are some that are considered the most important. 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
Nephrotic syndrome/CKD
Hyperhomocysteinemia or hemoglobinuria
Rheumatologic disease
Oral contraception or other medications
Myeloproliferative disorders
Being pregnant

Treatment for thrombus developing in any of these disorders is anticoagulation. The type and duration will depend on the site and the recurrence of the thromboses. 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 thromboses, thrombolytic therapy may also be suggested. This normalizes the blood flow much sooner.

Coding Concerns and Facts:

So, now that we know a little more about what a hypercoagulable state is, let’s look at some of the coding concerns that may arise.

  • The diagnosis of hypercoagulable state is documented, is it coded? That all depends on if it meets one of the criteria for reporting a diagnosis in ICD-10-CM. Chances are, the patient is under medical treatment for the condition or monitoring and follow up is normally required. If documentation is unclear, a query would be needed to clarify if this was clinically significant on the current admission. Most often, primary hypercoagulable state will be reported as these don’t go away. For the secondary hypercoagulable state, if the acquired disorder resolves, so may the hypercoagulable state in most patients.
  • Documentation shows that the patient has a venous thrombosis. Does this support coding the thrombosis and also the hypercoagulable state? NO! Since there is a thrombus wouldn’t that mean that the patient has a hypercoagulable state? NO! The physician must document the diagnosis before it can be coded. No assumptions can be made. Not all patients with a thrombosis have a hypercoagulable state or disorder.
  • If labs show that a patient has a prolonged prothrombin time, is this the same as hypercoagulable state? No!
  • Bleeding from anticoagulation therapy (administered correctly) is an adverse effect and NOT a complication.
  • Primary hypercoagulable state = inherited disorders
  • Secondary hypercoagulable state = primarily acquired disorders
  • If only hypercoagulable state is diagnosed, can the coder look at the patient’s other diseases/diagnoses and determine by that if they have primary or secondary? NO! Only the physician can make the link between the cause/effect.
  • Knowing the cause of the hypercoagulability determines the type and duration of treatment
  • The cause for hypercoagulable state can be correctly diagnosed in 80% to 90% of patients
  • The most common cause of acquired hypercoagulable state is antiphospholipid antibody syndrome
  • The most common cause of inherited hypercoagulable state is activated protein C resistance (factor V Leiden)
  • 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
  • Protein C and S deficiencies are vitamin K dependent
  • Thrombophilia is another term for hypercoagulable state
  • Testing for specific causes of hypercoagulability can be very costly
  • Remove or avoid risk factors that contribute to the hypercoagulable state (smoking, stop oral birth control, avoid prolonged immobilization, etc.,)
  • Even the inherited hypercoagulability disorders can be influenced by environmental factors and habits

For coding, it is all about clear, consistent, and concise documentation to arrive at the appropriate codes. There are a lot of disorders and diseases that can be responsible, but it is up to the physician to link and clarify this documentation. Sequencing of the codes, will depend on circumstances of admission and official coding guidelines for selection of the principal diagnosis.

Remember: Developing clots in the blood is not always a negative thing. Our blood should clot when injured, but not when just running through our bodies.

ICD-10-CM/PCS Coding Clinic,  Second Quarter 2021 Pages: 8-10
ICD-10-CM/PCS Coding Clinic, Fourth Quarter 2011 Pages: 93-95
ICD-10-CM/PCS Coding Clinic, Fourth Quarter 2008 Pages: 100-101
ICD-10-CM/PCS Coding Clinic, Third  Quarter 2008Pages: 16-17
ICD-10-CM/PCS Coding Clinic, Second Quarter 2017 Pages: 8-9
ICD-10-CM/PCS Coding Clinic, Fourth Quarter 2003 Pages: 56-57
ICD-10-CM Official Guidelines for Coding and Reporting FY 2021

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The information contained in this coding advice is valid at the time of posting. Viewers are encouraged to research subsequent official guidance in the areas associated with the topic as they can change rapidly. 

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