Red cell membrane disorders
Disorder . | Severity . | Inheritance . | Molecular defects . | Morphology . | Osmotic fragility . | Splenectomy . |
---|---|---|---|---|---|---|
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 |
Disorder . | Severity . | Inheritance . | Molecular defects . | Morphology . | Osmotic fragility . | Splenectomy . |
---|---|---|---|---|---|---|
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.