Erythrocyte lipid loss in hereditary spherocytosis.

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Erythrocyte lipid loss in hereditary spherocytosis.

Probable manifestations of the intrinsic corpuscular defect leading to shortened red blood cell (RBC) survival in hereditary spherocytosis (HS) have been induced by in vitro incubation of these erythrocytes (2-4). The classical in vitro changes that have been described are a marked increase in autohemolysis in the absence of metabolized substrate and an increase in osmotic fragility. Young, Izz...

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Erythrocyte energy metabolism in hereditary spherocytosis.

The incorporation of extracellular orthophosphate-(32)P into cellular ATP, 2,3-diphosphoglyceric acid, and inorganic phosphate has been measured over a period of 6 hours in vitro in red blood cells from normal subjects and from patients with hereditary spherocytosis who had undergone splenectomy. The pattern of labeling of the intracellular compounds was found to be the same in both types of re...

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Erythrocyte cellular and membrane deformability in hereditary spherocytosis.

In order to determine whether the relative rigidity of the hereditary spherocytosis (HS) red cell is due to membrane rididity or merely to an altered surface/volume ratio, we investigated the deformability of resealed red cell membranes from patients with HS. Whereas the osmotic fragility of intact red cells of HS patients showed the expected increase, the osmotic fragility of resealed HS membr...

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Increased erythrocyte adhesion in mice and humans with hereditary spherocytosis and hereditary elliptocytosis.

Mice with disruptions of the red blood cell (RBC) cytoskeleton provide severe hemolytic anemia models in which to study multiorgan thrombosis and infarction. The incidence of cerebral infarction ranges from 70% to 100% in mice with alpha-spectrin deficiency. To determine whether mutant RBCs abnormally bind adhesive vascular components, we measured adhesion of mouse and human RBCs to immobilized...

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Hereditary spherocytosis.

Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most patients having a well-compensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1966

ISSN: 0021-9738

DOI: 10.1172/jci105392