Hb-SS disease with splenic sequestration

This code identifies a severe form of sickle cell anemia (Hb-SS disease) complicated by acute splenic sequestration. Splenic sequestration is a life-threatening complication where a large amount of blood pools in the spleen, leading to a sudden drop in hemoglobin and potential hypovolemic shock. It is most common in young children with sickle cell disease.

Use this code when documentation clearly indicates a diagnosis of homozygous sickle cell disease (Hb-SS) and an acute episode of splenic sequestration. This typically presents with a rapidly enlarging spleen, pallor, and signs of anemia or shock in a patient with known sickle cell disease. Supporting documentation should include laboratory findings (e.g., acute drop in hemoglobin) and clinical assessment of splenic enlargement.

• Always sequence this code first if it is the primary reason for the encounter, as it represents an acute, life-threatening complication.
• Do not use this code for other types of sickle cell disease (e.g., Hb-SC, sickle-beta thalassemia) or for chronic splenic issues without acute sequestration.
• Ensure documentation specifies "Hb-SS" and "splenic sequestration" to support this specific code.