Other sickle-cell disorders with splenic sequestration

This code signifies a variant of sickle cell disease, other than sickle cell anemia, that is complicated by acute splenic sequestration. Splenic sequestration is a severe and potentially life-threatening complication where a large volume of blood pools in the spleen, leading to a sudden drop in hemoglobin and an enlarged, tender spleen.

Apply this code when documentation specifies a sickle cell disorder (e.g., sickle-hemoglobin C disease, sickle beta-thalassemia) and explicitly states the presence of splenic sequestration. This typically occurs in pediatric patients but can affect adults. Documentation should clearly indicate the acute nature of the sequestration event.

• Ensure the documentation specifies "other" sickle cell disorder, not sickle cell anemia (D57.0-).
• Verify that splenic sequestration is explicitly documented as a complication, not just splenomegaly.
• Sequence this code as the principal diagnosis when the splenic sequestration is the primary reason for the encounter.