Sickle-cell thalassemia beta plus with acute chest syndrome
ICD-10 D57.451 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta plus with acute chest syndrome.
Sickle-cell thalassemia beta plus is a complex hemoglobinopathy resulting from the combination of sickle cell disease and beta-thalassemia. This condition is characterized by the presence of both sickle hemoglobin (HbS) and reduced production of beta-globin chains, leading to a mixed phenotype of sickle cell disease and thalassemia. Acute chest syndrome (ACS) is a severe complication of sickle cell disease, presenting with chest pain, fever, and respiratory symptoms, often due to pulmonary vaso-occlusion, infection, or fat embolism. Patients with D57.451 may experience recurrent episodes of ACS, which can lead to significant morbidity and mortality. Management typically involves supportive care, pain management, and treatment of underlying causes, such as antibiotics for infections. Genetic factors play a crucial role in the severity and frequency of complications, as the specific mutations in the beta-globin gene can influence the clinical presentation and response to treatment.
Detailed patient history, genetic testing results, and treatment plans.
Management of acute chest syndrome, blood transfusions, and hydroxyurea therapy.
Ensure documentation reflects the severity of symptoms and treatment responses.
Pulmonary function tests, imaging studies, and treatment for respiratory symptoms.
Evaluation of respiratory distress in patients with sickle cell disease.
Document any acute exacerbations and their management thoroughly.
Used during hospitalization for acute chest syndrome management.
Document the reason for transfusion and patient response.
Hematology specialists should ensure accurate coding of transfusion episodes.
Coding D57.451 accurately reflects the complexity of managing patients with both sickle-cell disease and thalassemia, particularly when acute complications arise, ensuring appropriate treatment and reimbursement.
