Hereditary factor VIII deficiency
Chapter 3:Diseases of the blood and blood-forming organs
ICD-10 D66 is a billable code used to indicate a diagnosis of hereditary factor viii deficiency.
Hereditary factor VIII deficiency, also known as hemophilia A, is a genetic bleeding disorder caused by a deficiency of clotting factor VIII, which is essential for normal blood coagulation. This condition is typically inherited in an X-linked recessive pattern, primarily affecting males, while females may be carriers. Patients with this deficiency experience prolonged bleeding episodes, particularly after injuries or surgeries, and may also have spontaneous bleeding into joints and muscles. The severity of the condition can vary widely, with some individuals experiencing mild symptoms and others suffering from severe bleeding complications. Diagnosis is usually confirmed through laboratory tests that measure factor VIII levels and assess the patient's coagulation profile. Management of hereditary factor VIII deficiency often involves factor replacement therapy, which can be administered on-demand during bleeding episodes or as prophylaxis to prevent bleeding. Patients may also require education on avoiding activities that could lead to injury and regular monitoring of factor levels to tailor treatment effectively.
Detailed history of bleeding episodes, family history, and laboratory results.
Management of acute bleeding episodes, prophylactic treatment planning, and patient education.
Ensure documentation reflects the severity of the deficiency and treatment response.
Growth and development assessments, family history, and vaccination status.
Routine monitoring of children with hemophilia, management of bleeding during procedures.
Consider age-related factors in treatment plans and education for families.
Used for routine monitoring of factor levels in patients with hemophilia.
Document the reason for blood draw and any relevant clinical history.
Hematology specialists should ensure that lab results are clearly linked to the patient's treatment plan.
Hereditary factor VIII deficiency is primarily caused by mutations in the F8 gene, which is responsible for producing clotting factor VIII. This genetic defect leads to insufficient levels of the factor, resulting in impaired blood coagulation.
