The Red Blood Cell In Thalassemia

Eliezer A. Rachmilewitz, Ariella Oppenheim, Oded Shalev

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

A common denominator in all of the thalassemic disorders is the reduced production of one or more of the globin chains of the hemoglobin molecules within the red blood cell (RBC). The mature RBC of a human adult contains a heterogenous mixture of the hemoglobins. The morphological appearance of thalassemic RBC is grossly abnormal. Since the amount of hemoglobin A per cell is less than normal, the cells appear poorly hemoglobinized displaying hypochromia and microcytosis, reminiscent of the RBC found in profound iron deficiency anemia. Whereas light microscopy provides morphological evidence of the abnormal shape of thalassemic RBC, transmission electron-microscopy has added additional morphological information at the ultrastructural level and contributed to our understanding of the pathophysiology of the disease. Exogenous oxidative stress resulted in crosslinking of several membrane proteins particularly in the thalassemic RBC when compared to control RBC.
Original languageAmerican English
Title of host publicationGenetically Abnormal Red Cells
Editors R.L. Nagel
Place of PublicationBoca Raton
PublisherCRC Press
Pages137-160
Volume1
ISBN (Electronic)9780429263989
StatePublished - 1988

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