Sickle-cell thalassemia beta plus with acute chest syndrome

This code signifies a specific type of hemoglobinopathy where an individual has inherited both sickle cell trait and beta thalassemia, complicated by the acute onset of chest pain, fever, and pulmonary infiltrates. This combination results in a more severe clinical presentation than sickle cell trait alone, with the added risk of vaso-occlusive crises affecting the lungs.

Assign this code when documentation clearly indicates a diagnosis of sickle-cell beta thalassemia plus, and the patient is experiencing acute chest syndrome. This typically involves findings such as new pulmonary infiltrates on chest imaging, along with symptoms like dyspnea, cough, or chest pain in a patient with this specific hemoglobinopathy.

• Ensure documentation specifies "beta plus" to differentiate from other forms of sickle-cell thalassemia.
• Verify that acute chest syndrome is explicitly diagnosed, not just suspected or ruled out.
• Do not confuse with codes for sickle cell anemia without thalassemia or other forms of thalassemia without sickle cell.