D58.0

HCC Category Mapping

What This Code Means

A genetic blood disorder where red blood cells are abnormally shaped like spheres, making them fragile and prone to breaking down prematurely, leading to anemia.

Coding Tips

• •This is an inherited condition; document family history when available
• •Specify if complications like gallstones or spleen enlargement are present

Clinical Significance

Hereditary spherocytosis is the most common inherited hemolytic anemia in people of Northern European descent, caused by defects in red blood cell membrane proteins (spectrin, ankyrin, band 3, or protein 4.2) that result in spherically shaped, fragile red blood cells. These abnormal cells are selectively trapped and destroyed in the spleen, leading to chronic hemolytic anemia of variable severity. Complications include gallstone formation from chronic bilirubin excess, aplastic crises from parvovirus B19 infection, and splenomegaly.

Documentation Requirements

• ✓Document the hereditary nature of the condition with family history when available.
• ✓Record peripheral blood smear findings showing spherocytes and osmotic fragility test results.
• ✓Include hemoglobin level, reticulocyte count, indirect bilirubin, lactate dehydrogenase, and haptoglobin values.
• ✓Document disease severity (mild, moderate, or severe), spleen size, history of gallstones, and whether splenectomy has been performed or is planned.

Commonly Confused Codes