Hereditary spherocytosis

This code represents a genetic disorder characterized by defects in red blood cell membrane proteins, leading to fragile, spherical red blood cells (spherocytes) that are prematurely destroyed in the spleen. This results in chronic hemolytic anemia, often accompanied by jaundice, splenomegaly, and gallstones.

Use this code for patients diagnosed with hereditary spherocytosis, confirmed by laboratory findings such as increased osmotic fragility, spherocytes on peripheral blood smear, and often a positive family history. It is appropriate for both initial diagnosis and ongoing management of the condition.

• Ensure documentation specifies "hereditary" spherocytosis to differentiate from acquired forms.
• Consider co-reporting codes for associated conditions like splenomegaly (R16.1), cholelithiasis (K80.-), or anemia (D64.9 if not otherwise specified as hemolytic).
• Do not confuse with other types of hemolytic anemia; confirm the specific diagnosis of hereditary spherocytosis.