Sickle-cell thalassemia beta plus with cerebral vascular involvement
ICD-10 D57.453 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta plus with cerebral vascular involvement.
Sickle-cell thalassemia beta plus is a complex hemoglobinopathy characterized by the presence of both sickle hemoglobin (HbS) and thalassemia, specifically beta-thalassemia. This condition leads to a reduction in the production of normal hemoglobin, resulting in chronic hemolytic anemia. Patients with this disorder often experience vaso-occlusive crises due to the sickling of red blood cells, which can lead to various complications, including acute chest syndrome and stroke. Cerebral vascular involvement indicates that the patient has experienced cerebrovascular accidents (CVAs) or transient ischemic attacks (TIAs) as a result of the sickling process, which can compromise blood flow to the brain. The interplay of genetic factors, including mutations in the HBB gene responsible for beta-globin production, contributes to the severity of the disease. Management typically involves supportive care, pain management, and monitoring for complications, with potential interventions such as blood transfusions or hydroxyurea therapy to reduce sickling events.
Detailed history of hemolytic anemia, laboratory results showing hemoglobin levels, and evidence of cerebral vascular events.
Management of vaso-occlusive crises, monitoring for stroke symptoms, and treatment planning for chronic anemia.
Ensure documentation reflects the complexity of the disease and any interventions performed.
Neurological assessments, imaging studies (e.g., MRI, CT) showing evidence of stroke or TIA.
Evaluation of stroke symptoms in patients with sickle-cell thalassemia, management of neurological complications.
Document the relationship between sickle cell disease and neurological findings clearly.
Used during hospitalization for severe anemia management.
Document the reason for transfusion and patient’s hemoglobin levels.
Hematology specialists should ensure accurate coding of transfusion events.
Coding D57.453 accurately reflects the complexity of a patient's condition, including both sickle-cell disease and thalassemia, as well as any associated complications like cerebral vascular involvement, which is crucial for appropriate management and reimbursement.
