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Review : Disorders of the Red Cell MembraneA review of biochemical and physiologic altera tions of erythrocyte membranes which may lead to morphologic changes and shortened cell survivalDivision of Hematolpgy, Department of Pediatrics, UCLA School of Medicine, Los Angeles, California 90024 Morphologically similar cells can be seen in such varied conditions as liver disease, renal disease, a-betalipoproteinemia, malab sorption states, congenital nonspherocytic anemias, thalassemia, iron deficiency, pykno cytosis, hemolytic-uremic syndrome, erythro cyte enzyme deficiencies, etc. The common denominator in these condi tions may be an increase in membrane rigid ity, resulting from a diversity of causes. These would include altered protein-lipid- carbohydrate composition, abnormally struc tured hemoglobins, the presence of Heinz bodies due to altered glycolysis or unstable hemoglobins. The end result of such altered membrane elasticity, regardless of cause, would be fragmentation and destruction of the red cells during passage through small vascular channels, particularly in the spleen. Changes in erythrocyte stromal composi tion often seem to reflect more generalized systemic metabolic disorders. Whether any of the alterations of membrane composition are causally related to either the morpho logic changes or the shortened cell survival still remains to be proven.
Clinical Pediatrics, Vol. 7, No. 8,
451-464 (1968) |
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