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Definition

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.

Acronym

OHST

Synonyms

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

Keywords

Hereditary hemolytic anemia