Oncology & Hematology Coding Alert

What do 13 factors, hereditary conditions, and these Greek and Latin words have in common?

On the surface, diagnosis coding for blood coagulation disorders is pretty straightforward. After all, ICD-10-CM chapter 3 (Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)) is the only chapter in the whole of ICD-10-CM that has no chapter-specific guidelines.

That means you don’t have to worry about any special instructions affecting the way you assign a code from D65-D69 (Coagulation defects, purpura and other hemorrhagic conditions) other than the Excludes1, Use additional code, or Code first instructions that accompany the individual codes.

Even so, you should take note of a few things before definitively assigning a diagnosis code for a given coagulation condition.

Factor 1: Know Your Factors for Hemorrhagic Condition Codes

Proteins in the blood regulate the speed at which blood clots when you are cut or injured. If your blood clots too easily, you run the risk of developing thromboses, or blood clots, which will obstruct the flow of blood through your body, resulting in a potentially fatal deep vein thrombosis (DVT). If your blood doesn’t clot, on the other hand, you can bleed too much and hemorrhage.

Proteins, called coagulation factors, control the blood’s ability to clot; if your blood is deficient in these proteins, then it won’t clot properly. The proteins are known either by their name or by a Roman numeral. Altogether, you have twelve of them (pathologists now regard a thirteenth factor, VI, as belonging to another factor), and they are useful in helping you choose the correct ICD-10-CM code for the various hemorrhagic conditions.

Remember this: Factor VIII (antihemophilic) deficiency can be responsible for two different forms of hemophilia: hemophilia A, or classical hemophilia (D66), and Von Willebrand’s disease (D68.0). To distinguish between the two, you’ll need to confirm whether the Factor VIII deficiency is vascular, meaning the clotting protein binds to blood vessels, or functional.

Factor 2: Know What’s Hereditary Hemophilia and What’s Acquired

One possible source of confusion in coagulation disorder coding involves distinguishing between hereditary, or congenital, hemophilia, and acquired hemophilia.

“Congenital hemophilia is hereditary and is distinguished by a deficiency of certain clotting factors which occurs at birth,” whereas “acquired hemophilia is not hereditary. This is an autoimmune disorder,” says Leah Fuller, CPC, COC, Senior Consultant, Pinnacle Enterprise Risk Consulting Services LLC, Centennial, Colorado.

Acquired hemophilia, usually seen in adults, can develop consequent to illness, medications, or pregnancy. In this type of hemophilia, antibodies develop against clotting factors and these antibodies inactivate the clotting factors leading to bleeding. “This means the body mistakenly attacks healthy tissue and cells, specifically clotting factors, which in turn leads to excessive bleeding. In some cases of acquired hemophilia, the patient has an underlying issue; in others, the cause is unknown,” Fuller continues.

To code acquired hemophilia accurately, you’ll need to bypass the codes for hemophilia A, B, or C and head straight to D68.311 (Acquired hemophilia), which your provider may also document as autoimmune or secondary hemophilia.

“The provider should document in the medical record the type of hemophilia the patient has,” Fuller cautions, so if you have any doubt over which form of hemophilia a patient has, you must query the physician in charge.

Factor 3: Know These Greek and Latin Words to Pinpoint Clotting Disorders

On the other side of the blood coagulation disorder spectrum lie the clotting disorders: thrombophilia, purpura, and thrombocytopenia. Knowing that the Greek prefix, “thrombo,” means lump or clot, and that the word “purpura” is Latin for purple (as in the purple spots on the skin caused by leaking blood vessels) will help you pinpoint codes quickly and easily.

Of particular coding note among the purpura conditions are D69.1 (Qualitative platelet defects), which carries the synonyms of thromboasthenia (hemorrhagic) (hereditary) and thrombocytopathy and has an Excludes1 instruction for von Willebrand’s disease (D68.0), and D69.3 (Immune thrombocytopenic purpura).

Like hemophilia, you’ll find the same distinction between hereditary or acquired thrombophilia and thrombocytopenia, only here the different conditions are identified as primary (hereditary) or secondary (acquired). So, you should code primary thrombophilia with D68.5- (Primary thrombophilia), while you should report secondary thrombophilia with D68.69 (Other thrombophilia). For thrombocytopenia, the “Other primary thrombocytopenia” category D69.4- has the specific code option D69.42 (Congenital and hereditary thrombocytopenia purpura), with synonyms congenital thrombocytopenia and hereditary thrombocytopenia listed below it, and secondary thrombocytopenia options under D69.5- (Secondary thrombocytopenia).