Alpha thalassemia

This code signifies a severe form of alpha thalassemia, a genetic blood disorder characterized by a significant reduction or absence of alpha globin chains, leading to severe anemia. It typically presents as hemoglobin Bart's hydrops fetalis, a life-threatening condition in utero or shortly after birth.

Use this code for documented cases of severe alpha thalassemia, often identified prenatally or in neonates presenting with hydrops fetalis due to this genetic defect. Documentation should clearly indicate the diagnosis of alpha thalassemia, excluding trait or asymptomatic forms.

• Carefully differentiate from alpha thalassemia trait/minor (D56.3) and asymptomatic alpha thalassemia (D56.3) as these are Excludes1.
• Ensure documentation specifies the severity of alpha thalassemia to justify this code over less severe forms.
• Do not use for hydrops fetalis due to other causes (P56.0, P83.2), as these are also Excludes1.