Sickle-cell thalassemia beta plus with crisis
ICD-10 D57.45 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta plus with crisis.
Sickle-cell thalassemia beta plus with crisis is a complex hemolytic anemia resulting from the combination of sickle cell disease and beta-thalassemia. This condition arises due to mutations in the HBB gene, leading to abnormal hemoglobin production. Patients with this disorder experience episodes of vaso-occlusive crises, characterized by severe pain due to the sickling of red blood cells, which obstructs blood flow in small vessels. The clinical presentation may include symptoms of anemia, such as fatigue, pallor, and jaundice, alongside acute pain episodes. Management often involves pain control, hydration, and blood transfusions during crises. Long-term care may include hydroxyurea therapy to reduce the frequency of crises and improve overall quality of life. Genetic counseling is recommended for affected individuals and their families to understand inheritance patterns and reproductive options.
Detailed clinical history, laboratory results, and treatment plans must be documented.
Management of pain crises, blood transfusions, and hydroxyurea therapy.
Ensure documentation reflects the severity and frequency of crises for accurate coding.
Genetic testing results and family history must be clearly documented.
Genetic counseling for affected families and prenatal testing discussions.
Documentation should include inheritance patterns and implications for family members.
Used during acute management of severe anemia in patients with D57.45.
Document the indication for transfusion and patient response.
Hematology specialists should ensure compliance with transfusion protocols.
The 'with crisis' designation indicates that the patient is experiencing acute complications related to their sickle-cell thalassemia beta plus, which requires specific management and coding.