Coagulation disorders are disorders of the blood clotting factors that disrupt the body's ability to control blood clotting, resulting in either abnormal bleeding or excessive blood clotting. Either can be hereditary or acquired. The general terms “hypo-coagulopathy” and “hyper-coagulopathy” are defined as an increased tendency toward bleeding and an increased tendency toward blood clotting (thrombosis), respectively.

Primary (Hereditary) Hypercoagulable State, or Primary Thrombophilia, is caused by several rare genetic abnormalities. Factor V Leiden is the most common; others include elevated Factor VIII, and deficiencies in protein S, protein C and antithrombin. As with acquired thrombophilia, it is not typically identified until a thrombosis occurs, but can be diagnosed with various blood tests, such as lupus anticoagulant (LA) panels, activated protein C resistance, protein C and protein S activity, antithrombin activity, and genetic tests.

Secondary (Acquired) Hypercoagulable State, or Acquired Thrombophilia, are due to underlying systemic diseases or clinical conditions and cannot be identified until a thrombosis occurs.  

Acquired thrombophilia has many causes or precipitating factors such as:

• Immobilization (due to hospitalization, travel, obesity, stroke, etc.): Local venous stasis by accumulation of clotting factors and fibrin resulting in blood clot formation.
• Adverse effect of drugs: steroids, oral contraceptives, anticoagulants, heparin
• Clinical conditions: Major trauma, pregnancy, malignancy, diabetes, myeloproliferative disorders
• Autoimmune disease: Lupus anticoagulant and antiphospholipid syndrome (APS)

Patients who are “high risk” for thrombosis cannot be diagnosed with a hypercoagulable state until a thrombosis occurs. Likewise, if a thrombosis occurs due to immobilization, adverse effect of drugs, trauma, pregnancy, etc. and the precipitating factor is no longer present, the patient is no longer in a hypercoagulable state.  

Treatment of thrombosis and acquired thrombophilias such as lupus anticoagulant and APS involves anticoagulants like warfarin, heparin, direct factor Xa inhibitors (rivaroxaban, apixabn, and edoxaban), and and direct thrombin inhibitors (dabigatran).

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ICD-10 Coding of Hereditary and Acquired Thrombophilia

A 2021 Coding Clinic advised to assign code D68.69, Other thrombophilia, for a provider diagnosis of “secondary hypercoagulable state.” The case described a 79-year-old with a history of paroxysmal atrial fibrillation on anticoagulant maintenance who is diagnosed with secondary hypercoagulable state.

A hypercoagulable state in a patient on chronic anticoagulant therapy for atrial fibrillation would be extremely rare, since this is the direct opposite reaction of anticoagulant therapy, which is to prevent hyper-coagulopathy (thrombosis). Hypo-coagulopathy is the therapeutic effect of anticoagulant therapy, and abnormal bleeding is a common adverse effect (code D68.32).

Do not query for secondary hypercoagulable state in patients on anticoagulant therapy, unless paradoxical thrombosis occurs, and the anticoagulant therapy is discontinued.

The diagnosis of acquired thrombophilia depends on the clinical presentation described above.

COVID-19 and Hypercoagulable State

Recent clinical studies have found a high incidence of pulmonary embolism (PE) and deep vein thrombosis (DVT) in COVID-19 patients.

The development of hypercoagulability (PE and DVT) with COVID-19 is not well understood. Individuals with COVID-19 may have some complex coagulation abnormalities that create a hypercoagulable state but these cannot be recognized unless thrombosis occurs.

Circulating prothrombotic factors have been reported or suggested in patients with severe COVID-19 including elevated Factor VIII and fibrinogen levels. Vascular endothelial inflammation and injury may also contribute to the thrombotic tendency.

From a coding perspective, when thrombosis occurs in a patient with a recent COVID-19 infection, coding depends on whether there is an active COVID-19 infection or it has resolved. With an active infection, U07.1, COVID-19, is assigned first followed by all the complications such as the type and location of thrombo-embolism and the underlying hypercoagulable state if documented.

When a hypercoagulable state due to COVID-19 occurs after the active infection has resolved, the reason for admission (e.g., DVT and/or PE) is sequenced first followed by U09.9, post-COVID condition.

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