Increased Permeability of Erythrocyte Membrane in Thalassemia

Author:

CIVIDALLI GABRIEL12,LOCKER HILA13,RUSSELL ALEX14

Affiliation:

1. Department of Pediatrics and Child Health, Pediatric Research Laboratory and Hematologic Service, Hadassah University Hospital and Hebrew University—Hadassah Medical School, Jerusalem, Israel.

2. Department of Pediatrics, Pediatric Research Laboratory and Hematologic Service, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem, Israel.

3. Hadassah University Hospital, Jerusalem, Israel.

4. Hebrew University-Hadassah Medical School; Director of the Department of Pediatrics and Child Health, Hadassah University Hospital, Jerusalem, Israel.

Abstract

Abstract Increased sodium efflux is demonstrated in vitro in thalassemic red blood cells (and in those of patients with some other hemolytic conditions). This increase, in the presence of either normal or augmented sodium accumulation, must reflect an increased sodium leak into the cells. In vivo, the intracellular content of sodium and potassium is nearly normal. In vitro, however, thalassemia major RBC incubated for 24 hr undergo increased sodium gain, with somewhat more potassium loss. The resultant loss of cations presumably leads to intracellular dehydration. The converse obtains in hereditary spherocytosis, insofar as a sodium gain greater than the potassium loss leads to accumulation of water. It is concluded that increased permeability of the erythrocyte membrane often reflects an underlying hemolytic process. It is postulated that inability to maintain a normal composition of cations and water plays a part in the pathogenesis of hemolysis in thalassemia.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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