Coagulation Defects and Hemophilia ICD-10-CM codes

Hereditary bleeding disorders each have a code: D66 hemophilia A (factor VIII deficiency), D67 hemophilia B (factor IX deficiency), D68.1 factor XI deficiency (hemophilia C), and D68.2 other hereditary factor deficiencies. Von Willebrand disease is subdivided in D68.0-: D68.01 type 1, D68.020 to D68.029 the type 2 variants, D68.03 type 3, D68.04 acquired, and D68.00 when the type is not documented. Severity of hemophilia is not captured in the code.

D68.3- covers hemorrhagic disorders due to circulating anticoagulants. D68.32 (due to extrinsic circulating anticoagulants) is the code for bleeding caused by warfarin, heparin, or a direct oral anticoagulant, and it requires both a code for the bleeding site (for example K92.2 or I62.9) and T45.515A for the adverse effect. An elevated INR in an anticoagulated patient with no bleeding is R79.1 (abnormal coagulation profile), not D68.32. Z79.01 records long-term anticoagulant use. D68.311 is acquired hemophilia from autoantibodies, and D68.4 is acquired factor deficiency from liver disease or vitamin K deficiency.

Thrombophilia is grouped in D68.5- and D68.6-: D68.51 activated protein C resistance (factor V Leiden), D68.52 prothrombin gene mutation, D68.59 other primary thrombophilia, D68.61 antiphospholipid syndrome, D68.62 lupus anticoagulant syndrome, and D68.69 other thrombophilia. These are hypercoagulable states rather than bleeding disorders; code any current thrombosis (I82.-, I26.-) in addition and Z86.718 for history of venous thrombosis. Disseminated intravascular coagulation is D65. D68.9 (coagulation defect, unspecified) is for a documented coagulopathy with no cause identified and should not be used for an abnormal lab value alone.