Sickle-cell thalassemia beta plus with splenic sequestration

This code describes a specific type of sickle cell disease, where an individual has inherited both a sickle cell gene and a beta-thalassemia gene, resulting in a milder form of sickle cell anemia. The "plus" indicates some residual beta-globin production. The "splenic sequestration" specifies an acute, life-threatening complication where a large amount of blood pools in the spleen.

Use this code when documentation clearly indicates a diagnosis of sickle-cell beta plus thalassemia and an acute splenic sequestration crisis. This typically presents with sudden, severe anemia, splenomegaly, and hypovolemic shock. Supporting documentation includes hematology reports, physician notes detailing the specific thalassemia type, and clinical findings of splenic sequestration.

• Ensure the documentation specifies "beta plus" thalassemia, not "beta zero" or unspecified beta thalassemia.
• Confirm the presence of acute splenic sequestration; do not code for chronic splenomegaly or autosplenectomy.
• Sequence this code as the primary diagnosis when the splenic sequestration is the reason for the encounter.