D57.02

HCC Category Mapping

What This Code Means

Sickle cell disease with an acute crisis where the spleen traps and destroys red blood cells, causing severe anemia and organ dysfunction.

Coding Tips

• •Document evidence of splenic sequestration such as splenomegaly, acute anemia, or reticulocytosis in crisis
• •Ensure crisis status is documented; this code specifically indicates acute splenic sequestration complication

Clinical Significance

Splenic sequestration in Hemoglobin SS disease is a medical emergency where large volumes of blood become trapped in the spleen, causing rapid splenic enlargement, acute severe anemia, and potentially hypovolemic shock. It is most common in children under age 5 with Hemoglobin SS disease before autosplenectomy occurs but can also affect adults with other sickle cell genotypes. Mortality rates are high without prompt transfusion and supportive care.

Documentation Requirements

• ✓Documentation must confirm Hemoglobin SS genotype and the diagnosis of splenic sequestration with evidence of acute splenomegaly, rapid hemoglobin decline (typically >2 g/dL from baseline), reticulocytosis, and clinical signs of acute anemia or hemodynamic instability.
• ✓Record physical exam findings of splenic enlargement, vital signs, laboratory values including hemoglobin drop, platelet count, and reticulocyte count.
• ✓Document treatment including transfusion volumes, and note if splenectomy is performed or recommended.

Commonly Confused Codes