D68.311
BillableAcquired hemophilia
HCC Category Mapping
V28HCC 112 — Von Willebrand Disease and Other Coagulation Defects
0.247V24HCC 48 — Coagulation Defects and Other Specified Hematological Disorders
0.209ESRDHCC 48 — Coagulation Defects and Other Specified Hematological Disorders
0.000What This Code Means
A bleeding disorder that develops when the body produces antibodies against clotting factor VIII, preventing normal blood clotting.
Coding Tips
- •Confirm this is acquired (not congenital hemophilia A) and document the presence of factor VIII inhibitors
- •Note any associated conditions such as autoimmune disorders, malignancy, or recent factor VIII exposure that may have triggered antibody formation
Clinical Significance
Acquired hemophilia is a rare autoimmune disorder caused by spontaneous development of autoantibodies (inhibitors) against factor VIII, most commonly occurring in elderly patients, postpartum women, or in association with autoimmune diseases and malignancies. It presents with sudden-onset severe bleeding in a previously healthy individual, with a mortality rate of 9-22%. Unlike hereditary hemophilia, joint bleeding is uncommon while soft tissue and mucosal bleeding predominate.
Documentation Requirements
- ✓Document the presence of factor VIII inhibitor with titer level (Bethesda units), reduced factor VIII activity, prolonged aPTT that does not correct on mixing study, and underlying associated conditions.
- ✓Record the acute bleeding management strategy (bypassing agents such as FEIBA or rFVIIa) and immunosuppressive therapy used for inhibitor eradication.
Commonly Confused Codes
D66 (Hereditary factor VIII deficiency) — lifelong condition present from birth, unlike acquired hemophiliaD68.312 (Antiphospholipid antibody with hemorrhagic disorder) — different autoantibody mechanismD68.318 (Other hemorrhagic disorder due to intrinsic anticoagulants) — for inhibitors against other factors.