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An autosomal dominant disorder of red cell membrane permeability to monovalent cations, characterized by macrocytic hemolytic anemia of fluctuating severity, circulating erythrocytes with slit-like lucencies (stomata) evident on fixed, stained peripheral blood smears. OHST red cells exhibit cation leak, resulting in elevated cell sodium content with reduced potassium content. The disease is marked by splenomegaly or hepatosplenomegaly, cholelithiasis and a strong tendency for iron overload.




Hereditary, overhydrated, cation-leak stomatocytosis
Overhydrated cation leak stomatocytosis
Potassium sodium disorder of erythrocyte


Hereditary hemolytic anemia


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