Hereditary elliptocytosis

This code represents a rare, inherited disorder affecting red blood cells, causing them to be oval or elliptical in shape rather than the typical biconcave disc. This abnormal morphology can lead to increased red blood cell destruction (hemolysis), resulting in varying degrees of anemia, jaundice, and splenomegaly. The severity of symptoms is highly variable, ranging from asymptomatic carriers to severe hemolytic anemia requiring transfusions.

Use this code for patients diagnosed with hereditary elliptocytosis, confirmed through laboratory findings such as peripheral blood smear showing characteristic elliptical red cells, and often supported by family history. It is appropriate for both symptomatic and asymptomatic individuals identified with the condition. Documentation should clearly indicate the diagnosis of hereditary elliptocytosis.

• Do not confuse with hereditary spherocytosis (282.0), which involves spherical red blood cells and has distinct genetic and clinical features.
• Code any associated complications, such as anemia (e.g., 285.9 for unspecified anemia) or splenomegaly (e.g., 789.2), as secondary diagnoses.
• Ensure documentation specifies "hereditary elliptocytosis" to differentiate from acquired forms of elliptocytosis, though these are rare.