scholarly journals Physical Properties of Red Cells as Related to Effects in Vivo. III. Effect of Thermal Treatment on Survival of Red Cells in the Dog. Role of the Spleen

Blood ◽  
1968 ◽  
Vol 32 (6) ◽  
pp. 872-883 ◽  
Author(s):  
DENNIS J. CARLSON ◽  
THOMAS HALE HAM
Keyword(s):  
Sickle Cell Disease ◽  
Thermal Treatment ◽  
Peripheral Blood ◽  
Hereditary Spherocytosis ◽  
Osmotic Fragility ◽  
Red Cells ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

Abstract Dog red cells heated at 49 C. for 15 minutes with no change in osmotic fragility, showed a decreased rate of survival in vivo, and increased sequestration by the spleen, and an increase in the osmotic fragility when recovered from the spleen. The peripheral blood showed normal osmotic fragility at all times. These changes were comparable to those seen in the spleen in homozygous sickle-cell disease and hereditary spherocytosis. There was no hemoglobinemia. Splenectomy decreased the rate of destruction of such heated red cells in vivo. In these studies the rigidity of the red cells with increased viscosity, but with normal osmotic fragility, may have resulted in the removal of the heated red cells by the spleen and subsequent conditioning of these cells in the spleen. In contrast to the 15 minutes heating, dog cells heated at 49 C. for 60 minutes had increased osmotic fragility and were rapidly removed from the circulation. There was sequestration by the spleen or by the liver with hemoglobinemia. The red cells with the greatest increase in osmotic fragility were not preferentially removed in vivo.

scholarly journals Chronic leg ulceration in homozygous sickle cell disease: the role of venous incompetence

2002 ◽  
Vol 119 (2) ◽  
pp. 567-571 ◽  
Author(s):  
Andrea Clare ◽  
Michael FitzHenley ◽  
June Harris ◽  
Ian Hambleton ◽  
Graham R Serjeant
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

Regulation of Na+/Mg2+ Exchange in Sickle Erythrocytes By Endothelin-1

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4064-4064
Author(s):  
Pablo A. Rivera ◽  
Yaritza Inostroza ◽  
Jose R. Romero ◽  
Alicia Rivera
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Ex Vivo ◽  
Red Cells ◽  
Cell Disease ◽  
Magnesium Homeostasis ◽  
Increased Activity ◽  
Exchange Activity

Abstract Excess levels of endothelin-1 (ET-1), erythrocyte sickling and chronic inflammation have been proposed as important contributors to the pathophysiology of sickle cell disease (SCD). We have shown that ET-1 receptor antagonists improve hematological parameters by reducing Gardos channel activity in two transgenic mouse models of SCD while reducing oxidant stress by decreasing circulating levels of protein disulfide isomerase. Magnesium (Mg2+) deficiency, mediated in part via increased erythrocyte Na+/Mg2+ exchanger activity, has been demonstrated to contribute to erythrocyte dehydration, K+ loss and sickling in SCD. However, the relationship between ET-1 and the Na+/Mg2+ exchanger in SCD remains unclear. We measured Na+/Mg2+ exchange activity in ex vivo red cells and observed increased activity following in vitro incubation of human (2.2 ± 0.2 to 3.2 ± 0.1 mmol/1013 cell x h, P<0.03, n=5) and mouse red blood cells with ET-1 (P<0.001, n=5); events that were significantly blocked by pre-incubation of cells with 1 μM BQ788, a selective inhibitor of ET-1 type B receptors. In addition, in vitro deoxygenation of sickle red cells led to increased exchanger activity that was inhibited by impramine, a Na+/Mg2+ exchange inhibitor, and associated with reduced deoxygenation-stimulated sickle cell dehydration. These results suggest an important role for ET-1 and cellular magnesium homeostasis in sickle cell disease. To this end, we studied Na+/Mg2+ exchange activity in ex vivo erythrocytes from three transgenic sickle mouse models and observed increased activity in these cells when compared to red cells from either Hb A transgenic or C57BL/J6 wild-type mice (P<0.03, n=4). We then tested the in vivo effects of ET-1 receptor antagonists on erythrocyte Na+/Mg2+ exchange activity in the BERK mouse, a transgenic model of SCD. We blocked ET-1 receptors type A and B by in vivo treatment with BQ-788 and BQ-123 (360mg/Kg/Day) for 14 days and observed lower erythrocyte exchanger activity when compared to cells from vehicle treated BERK mice (P<0.02, n=6). Thus our results suggest that ET-1 receptor blockade represents an important therapeutic approach to control erythrocyte volume and magnesium homeostasis that may lead to improved inflammatory and vascular complications observed in SCD. Supported by NIH R01HL090632 to AR. Disclosures No relevant conflicts of interest to declare.

ROLE OF SPLENECTOMY IN HOMOZYGOUS SICKLE CELL DISEASE IN CHILDHOOD

The Lancet ◽  
1984 ◽  
Vol 323 (8368) ◽  
pp. 88-91 ◽  
Author(s):  
A.M. Emond ◽  
S. Venugopal ◽  
P. Morais ◽  
R.G. Carpenter ◽  
G.R. Serjeant
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease

2004 ◽  
Vol 75 (2) ◽  
pp. 63-72 ◽  
Author(s):  
Qin Zen ◽  
Milena Batchvarova ◽  
Christina A. Twyman ◽  
Christine E. Eyler ◽  
Huiling Qiu ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
High Density ◽  
Red Cells ◽  
Cell Disease

scholarly journals Cell-bound autologous immunoglobulin in erythrocyte subpopulations from patients with sickle cell disease

Blood ◽  
1985 ◽  
Vol 65 (5) ◽  
pp. 1127-1133 ◽  
Author(s):  
GA Green ◽  
MM Rehn ◽  
VK Kalra
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Red Cells ◽  
Cell Subpopulation ◽  
Red Cell ◽  
Cell Disease ◽  
Sickle Erythrocyte ◽  
Cell Subpopulations ◽  
Cell Fractions

Abstract Previously, we have demonstrated a parallel between most-dense (bouyant density) sickle erythrocyte subpopulations and most-dense aged normal red cells in the organization of membrane components in the intact cell. The present study has addressed the possibility that a corresponding similarity may exist between most-dense sickled red cell subpopulations and aged normal erythrocytes in the development of membrane protein components that function as receptors for autologous immunoglobulin (Ig). Autologous IgG retained by density-fractionated erythrocytes has been estimated by a nonequilibrium 125I-protein A (Staphylococcus aureus) binding assay. Results show that most-dense sickle cell fractions contain more (2.7-fold and 1.8-fold, P less than .005) cell-bound IgG in comparison to younger sickle erythrocyte fractions sedimenting at low density. Parallel findings were obtained after similar analyses of normal (homozygous-A) erythrocyte fractions. Detection of the presence of specific IgG was also carried out by direct binding of fluorescein isothiocyanate-conjugated anti-human IgG to density-separated red cell fractions followed by analyses of the fluorescent cell populations by flow cytometry. Results showed significantly higher levels of IgG bound to most-dense (12.1% +/- 2.5% and 8.8% +/- 0.5%-) sickle red cell subpopulations (P less than .005) in comparison to younger sickle erythrocyte fractions sedimenting at low densities (3.8% +/- 0.32% and 4.7% +/- 1.6% IgG-positive red cell subpopulation). These results indicate that some of the same membrane changes that occur at about 120 days in normal red cells are also apparent in the chronologically younger (life span in vivo, ten to 40 days) sickle erythrocyte. The increased retention of IgG by most-dense irreversibly sickled cell-enriched fractions in comparison to least- dense reversibly sickled cells or pre-irreversibly sickled erythrocyte fractions, suggests that alterations in the topography of the sickle cell membrane during the transformation in vivo to the most-dense irreversibly sickled cell morphology may produce the unmasking of cryptic antigenic sites. In addition, these findings may indicate that opsonization of specific erythrocyte subpopulations may play a role in the pathophysiology of sickle cell disease.

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