Sickle-cell thalassemia beta zero with splenic sequestration

This code describes a severe form of sickle cell disease where an individual has inherited one gene for sickle cell hemoglobin and one gene for beta-zero thalassemia, resulting in a complete absence of normal beta-globin production. The "splenic sequestration" component indicates an acute, life-threatening complication where a large amount of blood pools in the spleen, leading to a sudden drop in hemoglobin and potential hypovolemic shock.

Use this code when documentation clearly indicates a diagnosis of sickle cell-beta zero thalassemia and an acute episode of splenic sequestration. This typically presents with sudden pallor, left upper quadrant pain, splenomegaly, and a rapid decrease in hemoglobin. Supporting documentation would include hematology reports confirming the specific hemoglobinopathy and clinical notes detailing the acute splenic event.

• Ensure the documentation explicitly states "beta zero" thalassemia, not "beta plus" (D57.431) or unspecified (D57.439).
• This code inherently includes the splenic sequestration crisis; do not code an additional crisis code.
• Verify that the patient's genetic profile or hemoglobin electrophoresis results support the sickle cell-beta zero thalassemia diagnosis.