D56.1

HCC Category Mapping

What This Code Means

A genetic blood disorder where the body cannot make enough beta-globin chains, which are part of hemoglobin. This causes moderate to severe anemia and may require regular blood transfusions.

Coding Tips

• •Specify the type: beta thalassemia major, intermedia, or minor based on clinical severity
• •Document transfusion requirements and any complications like iron overload

Clinical Significance

Beta thalassemia results from mutations reducing or eliminating beta-globin chain production, causing ineffective erythropoiesis and hemolytic anemia. Beta thalassemia major (Cooley anemia) is transfusion-dependent from early childhood, while beta thalassemia intermedia presents with moderate anemia that may or may not require transfusions. The condition carries significant morbidity from iron overload, skeletal changes, and organ damage, making it a high-acuity chronic condition.

Documentation Requirements

• ✓Documentation must specify the beta thalassemia subtype (major, intermedia, or minor), supported by hemoglobin electrophoresis, genetic testing, or molecular analysis.
• ✓Record transfusion schedule and frequency, iron chelation therapy, ferritin levels, cardiac and hepatic iron assessment results, and complications including endocrinopathies, osteoporosis, or extramedullary hematopoiesis.
• ✓Annual comprehensive assessment documentation supports recapture.

Commonly Confused Codes