D66
BillableHereditary factor VIII deficiency
HCC Category Mapping
V28HCC 111 — Hemophilia
0.565V24HCC 46 — Severe Hematological Disorders
0.666ESRDHCC 46 — Severe Hematological Disorders
0.000What This Code Means
A hereditary bleeding disorder caused by deficiency or dysfunction of clotting factor VIII, commonly known as hemophilia A.
Coding Tips
- •Specify the severity level (mild, moderate, severe) if documented
- •Use additional codes for any complications such as inhibitor development
Clinical Significance
Hereditary factor VIII deficiency (Hemophilia A) is the most common severe inherited bleeding disorder, caused by deficient or defective clotting factor VIII. Severity correlates with factor VIII activity levels: severe (<1%), moderate (1-5%), or mild (5-40%). This condition requires lifelong factor replacement therapy and carries significant healthcare resource utilization with a high RAF weight.
Documentation Requirements
- ✓Document the severity level (severe, moderate, mild) based on factor VIII activity levels.
- ✓Record current treatment regimen including factor replacement products, dosing frequency, and whether the patient has developed inhibitors (antibodies to factor VIII).
- ✓Note any target joints, history of intracranial hemorrhage, and hepatitis/HIV status if applicable from prior blood product exposure.
Excludes 1 — Do NOT code together
- factor VIII deficiency with vascular defect (D68.0-)
Commonly Confused Codes
D67 (Hereditary factor IX deficiency/Hemophilia B) — clinically similar but different factor deficiency requiring different replacement productsD68.311 (Acquired hemophilia) — autoimmune condition developing later in life with autoantibodies against factor VIIID68.00-D68.09 (Von Willebrand disease) — may have low factor VIII but primary defect is in von Willebrand factor.