Table 2.

Red cell membrane disorders

DisorderSeverityInheritanceMolecular defectsMorphologyOsmotic fragilitySplenectomy
HS Mild to severe AD, AR, de novo Ankyrin-1, band 3, α-spectrin, β-spectrin, protein 4.2 Varying degree of spherocytes Mild to marked decrease Beneficial 
HE Nonhemolytic to severe AD α-spectrin, β-spectrin, protein 4.1 Elliptocytes and fragmented red cells Normal to marked decrease Beneficial 
OHS Mild to moderate AD RhAG Stomatocytosis Increased Not recommended 
HX Nonhemolytic to moderate AD Piezo-1, Gardos channnel Some target cells Decreased Not recommended 
DisorderSeverityInheritanceMolecular defectsMorphologyOsmotic fragilitySplenectomy
HS Mild to severe AD, AR, de novo Ankyrin-1, band 3, α-spectrin, β-spectrin, protein 4.2 Varying degree of spherocytes Mild to marked decrease Beneficial 
HE Nonhemolytic to severe AD α-spectrin, β-spectrin, protein 4.1 Elliptocytes and fragmented red cells Normal to marked decrease Beneficial 
OHS Mild to moderate AD RhAG Stomatocytosis Increased Not recommended 
HX Nonhemolytic to moderate AD Piezo-1, Gardos channnel Some target cells Decreased Not recommended 

AD, autosomal dominant; AR, autosomal recessive.

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