Yazarlar : Safeukui I, Buffet PA, Deplaine G, et al.

Yayın : Blood

Yayın Yılı : 2012

Pubmed Linki : http://www.ncbi.nlm.nih.gov/pubmed/22510876

Konu : Anemi

Literatür İçeriği :  

Abstract

Splenic sequestration of red blood cells (RBCs) with reduced surface area and cellular deformability has been long recognized to contribute to pathogenesis of several RBC disorders, including hereditary spherocytosis. However, the quantitative relationship between the extent of surface area loss and splenic entrapment remains to be defined. To address this issue, we perfused ex vivo normal human spleens with RBCs displaying varying degrees of surface area loss and monitored the kinetics of their splenic retention. Treatment with increasing concentrations of lysophosphatidylcholine resulted in a dose-dependent reduction of RBC surface area at constant volume, increased osmotic fragility and decreased deformability. The degree of splenic retention of treated-RBCs increased with increasing surface area loss. RBCs with a >18% average surface area loss (> 27% reduced S/V ratio) were rapidly and completely entrapped in the spleen. Surface-deficient RBCs appear to undergo volume loss after repeated passages through the spleen and escape from splenic retention. This study defines for the first time the critical extent of surface area loss leading to splenic entrapment and identifies an adaptive volume regulation mechanism that allows spherocytic RBCs to prolong their life span in circulation. This has significant implications for understanding the clinical heterogeneity of RBC membrane disorders.

Literatür Arşivi