Table 2.

Definition of congenital hemolytic diseases

Disease	ICD-10	Core criteria
Congenital
Thalassemia	D56	Positive hemoglobin electrophoresis/HPLC and/or gene test
Sickle cell anemia	D57	Positive hemoglobin electrophoresis/HPLC and confirmatory gene test
Hereditary spherocytosis	D588	Hemolysis + reticulocytosis + family history of hemolysis + negative antiglobulin test + increased osmotic fragility or positive EMA test.
Hereditary elliptocytosis	D581	Increased osmotic fragility, elliptocytes, and family history
Other hemoglobinopathies	D582	Positive hemoglobin electrophoresis/HPLC or gene test
Hereditary hemolytic anemia, unspecified	D589	Hemolysis + anemia + family history or congenital lifelong hemolysis and no better explanation available

Abbreviations: ICD-10, International Classification of Diseases Tenth Revision; HPLC, high-pressure liquid chromatography; EMA, eosin-5′-maleimide.