Sickle-cell thalassemia beta zero with cerebral vascular involvement
ICD-10 D57.433 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with cerebral vascular involvement.
Sickle-cell thalassemia beta zero is a complex hemolytic anemia resulting from the combination of sickle cell disease and beta-thalassemia. This condition is characterized by the production of abnormal hemoglobin, leading to the sickling of red blood cells, which can cause vaso-occlusive crises and chronic hemolysis. The 'beta zero' designation indicates that there is a complete absence of beta-globin chains, which exacerbates the severity of the disease. Cerebral vascular involvement refers to complications such as stroke or transient ischemic attacks (TIAs) that can occur due to the occlusion of blood vessels in the brain by sickled cells. Patients may present with neurological deficits, headaches, or seizures. Management often requires a multidisciplinary approach, including hematology, neurology, and possibly transfusion therapy to prevent further complications. Genetic counseling is also essential due to the hereditary nature of both sickle cell disease and thalassemia.
Detailed history of hemolytic anemia, genetic testing results, and treatment plans.
Management of vaso-occlusive crises, blood transfusions, and monitoring for stroke.
Ensure documentation reflects the severity of anemia and any neurological involvement.
Neurological assessments, imaging studies, and treatment plans for stroke or TIA.
Evaluation of stroke symptoms in patients with sickle cell disease.
Document the relationship between sickle cell disease and neurological symptoms.
Used during hospitalization for severe anemia or stroke prevention.
Document the indication for transfusion and patient response.
Hematology should ensure that the transfusion is justified based on hemoglobin levels.
The 'beta zero' designation indicates a complete absence of beta-globin chains, leading to more severe symptoms and complications compared to beta-thalassemia with some beta-globin production.
