Sickle-cell thalassemia
ICD-10 D57.4 is a billable code used to indicate a diagnosis of sickle-cell thalassemia.
Sickle-cell thalassemia is a genetic blood disorder that combines features of both sickle cell disease and thalassemia. It occurs when an individual inherits one sickle cell gene (HbS) from one parent and a thalassemia gene from the other. This condition leads to the production of abnormal hemoglobin, which can cause red blood cells to become rigid and sickle-shaped, leading to hemolytic anemia. Patients may experience episodes of pain due to vaso-occlusive crises, increased risk of infections, and complications related to chronic hemolysis. The severity of symptoms can vary widely depending on the specific type of thalassemia inherited (alpha or beta) and the proportion of sickle hemoglobin present. Management typically involves regular monitoring, pain management, and preventive measures against infections. Blood transfusions and hydroxyurea may be utilized to reduce complications and improve quality of life. Genetic counseling is also recommended for affected individuals and their families to understand inheritance patterns and reproductive options.
Detailed lab results, genetic testing, and treatment plans.
Management of pain crises, blood transfusions, and monitoring for complications.
Ensure clarity in documentation regarding the type of thalassemia and sickle cell status.
Family history, genetic counseling notes, and test results.
Counseling families about inheritance patterns and reproductive options.
Documentation must clearly outline genetic findings and implications for family members.
Used when a patient with sickle-cell thalassemia requires a blood transfusion due to severe anemia.
Document the indication for transfusion and the patient's hemoglobin levels.
Hematology specialists should ensure that the transfusion is justified based on clinical guidelines.
Sickle-cell thalassemia is a specific type of sickle cell disease that occurs when a person inherits both a sickle cell gene and a thalassemia gene. This results in a unique set of symptoms and complications that differ from those seen in individuals with sickle cell disease alone.