Oncology & Hematology Coding Alert

Time to put these new VWD, HIT, and HUS codes at your fingertips.

Changes to the D50-D89 (Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism) code range in ICD-10-CM 2023 are extensive. And Oct. 1, 2022 — the date they become effective for use — is not far away.

So, here’s a preview of all the major changes to help you hit the ground running.

Get Set for New von Willebrand Disease Type Codes

A slight change in name for this blood clotting condition from von Willebrand’s Disease (the removal of the apostrophe s) is the least important change from ICD-10-CM 2022. More important is the expansion of D68.0- (Von Willebrand disease) (VWD), which will now allow you to capture the specific type of the disorder as recognized by the Centers for Disease Control and Prevention (CDC) and others in the medical field (Source: https:/www.cdc.gov/ncbddd/vwd/facts.html).

Type 1: The most common form of the condition, in which the patient has low levels of either von Willebrand factor (VWF) or low levels of factor VIII (8), can now be coded with D68.01 (Von Willebrand disease, type 1).

Type 2: These subtypes, in which the patient’s VWF is sufficient but does not fulfill its function with blood platelets correctly, can now be described with the following codes:

• D68.020 (Von Willebrand disease, type 2A), where the VWF is not the sufficient size to enable the platelets to clot together
• D68.021 (Von Willebrand disease, type 2B), where the VWF attaches to platelets, but not at the right time
• D68.022 (Von Willebrand disease, type 2M), where the VWF does not correctly attach to the platelets
• D68.023 (Von Willebrand disease, type 2N), where the VWF attaches to the platelets correctly but not to the factor VIII (8) protein, which is also needed in the clotting process

You’ll also be able to use D68.029 (Von Willebrand disease, type 2, unspecified) if your provider does not specify the exact type 2 subtype of VWD.

“The four VWF type 2 qualitative deficiency variants, as reported by these new codes, will require additional information from the physician to identify and report the correct one. While it is always important to report the most specific code, the patient’s treatment for one deficiency type over another may be needed to justify a patient’s care regimen,” suggests Kelly C. Loya, CPC-I, CHC, CPhT, CRMA, associate partner, Pinnacle Enterprise Risk Consulting Services LLC, Centennial, Colorado.

Type 3: In this type of VWD, there is a “near complete absence of VWF, which makes type 3 the most severe form of VWD,” according to Amy Pritchett, CCS, CPC-I, CPMA, CDEO, CASCC, CANPC, CRC, CDEC, CMPM, C-AHI, senior consultant at Pinnacle Enterprise Risk Consulting Services LLC, Centennial, Colorado. Type 3 VWD is also very rare, which means you won’t be called upon to use new code D68.03 (Von Willebrand disease, type 3) often.

Make a note: The new VWD code group also contains D68.04 (Acquired von Willebrand disease) and D68.09 (Other von Willebrand disease), which includes platelet-type and pseudo-von Willebrand synonyms and a Code also instruction to use D69.1 (Qualitative platelet defects) “if applicable.”

Don’t Take the Hit and Miss the Heparin-Induced Thrombocytopenia Expansion

ICD-10-CM 2023 also features an overhaul of the D75.8- (Other specified diseases of blood and blood-forming organs) code group, most specifically an expansion of the heparin-induced thrombocytopenia (HIT) code D75.82-. Currently, you can only “report/assign HIT without the ability to define type/factors, but this expansion will allow for further specificity when coding for HIT,” says Pritchett.

Now, you’ll have to add a 6th character to report specific types of the dangerous condition, which can occur when a patient is placed on heparin, the blood-thinning medicine designed to prevent blood clots.

The codes in question are:

• D75.821 (Non-immune heparin-induced thrombocytopenia)
• D75.822 (Immune-mediated heparin-induced thrombocytopenia)
• D75.828 (Other heparin-induced thrombocytopenia syndrome)
• D75.829 (Heparin-induced thrombocytopenia, unspecified)

You’ll use D75.821 when your provider documents type 1 HIT, and D75.822 for type 2 HIT. And you’ll also have D75.828 to report autoimmune heparin-induced thrombocytopenia syndrome, delayed-onset or persisting heparin-induced thrombocytopenia. You’ll also have another new code, D75.84 (Other platelet-activating anti-PF4 disorders), which includes spontaneous heparin-induced thrombocytopenia syndrome (without heparin exposure).

Remember this: New ICD-10-CM Use additional code notes instruct you to use a code from T45.515- (Adverse effect of anticoagulants) when reporting D75.82-, and T50.B95- (Adverse effect of other viral vaccine) when reporting D75.84 when applicable.

Expand Your HUS Awareness

You may not know much about hemolytic-uremic syndrome (HUS) as the condition is somewhat rare, primarily affecting young children with compromised immune systems. Typically, they acquire the condition after being infected with the E. coli O57:H7 bacteria, but sometimes the cause is genetic, a complication from an organ transplant, the adverse effect of a drug, or unknown. But you should know about the four new codes to report it, and the extensive instructions for their use, in the event your provider has to treat an immunocompromised patient who has developed the disease.

The expansion of D59.3- (Hemolytic-uremic syndrome) makes D59.3 the parent code and will now include a specific code for typical HUS: D59.31 (Infection-associated hemolytic-uremic syndrome). The Use additional code instructions that previously existed under the parent code have moved to D59.31, and ICD-10-CM 2023 has greatly expanded the list of HUS-associated infections you will code to identify the cause of the HUS.

So, beginning Oct. 1, 2022, you are instructed to use a code such as B20 (Human immunodeficiency virus [HIV] disease), G00.1 (Pneumococcal meningitis), A40.3 (Sepsis due to Streptococcus pneumoniae), or B95.3 (Streptococcus pneumoniae as the cause of diseases classified elsewhere), in addition to the other infections moved from under D59.3- — B96.2- (Escherichia coli [E. coli ] as the cause of diseases classified elsewhere), J13 (Pneumonia due to Streptococcus pneumoniae), or A03.9 (Shigellosis, unspecified) — to identify the associated infection.

ICD-10-CM 2023 also introduces two other new codes under D59.3: D59.32 (Hereditary hemolytic-uremic syndrome) and D59.39 (Other hemolytic-uremic syndrome). You’ll use D59.39 for secondary HUS, which explains the extensive Code first, Code also, and Use additional code instructions that ICD-10-CM has introduced to accompany the codes. And for the whole D59.3- code group, you also have an instruction to Code also, if applicable, any associated acute kidney failure (N17.-) or chronic kidney disease (N18.-). Additionally, D59.30 (Hemolytic-uremic syndrome, unspecified) is available for use when the documentation does not support the underlying reason for HUS.

For the full list of 2023 ICD-10-CM updates, go to www.cms. gov/medicare/icd-10/2023-icd-10-cm.