scholarly journals Mechanical properties of sickle cell membranes

Blood ◽  
1990 ◽  
Vol 75 (8) ◽  
pp. 1711-1717 ◽  
Author(s):  
R Messmann ◽  
S Gannon ◽  
S Sarnaik ◽  
RM Johnson

Abstract The mechanical properties of sickle erythrocyte membranes were evaluated in the ektacytometer. When ghosts from the total red blood cell population were examined, the rigidity of the resealed ghosts and their rate of fragmentation by shear stress (t1/2) were normal. However, fractionation on Stractan density gradients revealed that sickle cells were heterogenous in their membrane mechanical properties. The ghosts from dense cell fractions exhibited both increased rigidity and decreased stability. Presumably, these altered mechanical properties are a reflection of the well-documented biochemical damage found in irreversibly sickle cell membranes. Nevertheless, neither of the alterations in mechanical properties are likely to be significant elements in the hemolysis of sickle cell anemia. Earlier studies of abnormal erythrocytes suggest that increases in membrane rigidity per se do not increase hemolysis, and they are, therefore, unlikely to do so in this case. The stability of membranes from the dense cell fractions was reduced to about two thirds of the control value. Comparison with the results of studies of red blood cell membranes with genetically defective or deficient spectrin suggests that a reduction in t 1/2 of 50% is not associated with significant increases in the rate of hemolysis. Although altered ghost stability and flexibility can be demonstrated in dense sickle cells, these changes in membrane mechanical properties are not likely to be significant factors in the hemolytic process.

Blood ◽  
1990 ◽  
Vol 75 (8) ◽  
pp. 1711-1717 ◽  
Author(s):  
R Messmann ◽  
S Gannon ◽  
S Sarnaik ◽  
RM Johnson

The mechanical properties of sickle erythrocyte membranes were evaluated in the ektacytometer. When ghosts from the total red blood cell population were examined, the rigidity of the resealed ghosts and their rate of fragmentation by shear stress (t1/2) were normal. However, fractionation on Stractan density gradients revealed that sickle cells were heterogenous in their membrane mechanical properties. The ghosts from dense cell fractions exhibited both increased rigidity and decreased stability. Presumably, these altered mechanical properties are a reflection of the well-documented biochemical damage found in irreversibly sickle cell membranes. Nevertheless, neither of the alterations in mechanical properties are likely to be significant elements in the hemolysis of sickle cell anemia. Earlier studies of abnormal erythrocytes suggest that increases in membrane rigidity per se do not increase hemolysis, and they are, therefore, unlikely to do so in this case. The stability of membranes from the dense cell fractions was reduced to about two thirds of the control value. Comparison with the results of studies of red blood cell membranes with genetically defective or deficient spectrin suggests that a reduction in t 1/2 of 50% is not associated with significant increases in the rate of hemolysis. Although altered ghost stability and flexibility can be demonstrated in dense sickle cells, these changes in membrane mechanical properties are not likely to be significant factors in the hemolytic process.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4383-4383
Author(s):  
Sabine Sipurzynski-Budraâ ◽  
Petra Sovinz ◽  
Gerhard Lanzer ◽  
Katharina Schallmoser

Abstract Abstract 4383 Background: Sickle cell disease, an autosomal recessive genetic blood disorder, is caused by abnormal hemoglobin (Hb) due to a substitution of valine by glutamic acid at β6 (HbS). Typical adverse events are vaso-occlusive crises due to obstruction of capillaries causing ischemia, pain and organ damage, as well as enhanced hemolysis and frequent hemolytic crises. Further complications may include acute chest syndrome and stroke. To prevent long-term sequelae, repeated blood transfusions are necessary to decrease erythropoiesis of sickle cells. We suggest that it may be more effective to reduce sickle cells by a red blood cell (RBC) exchange, which is advantageous in avoiding iron overload by transfusions. Patient and Methods: We report on a 10 year old boy suffering from the homozygote form of sickle cell anemia. RBC exchange was performed using the Spectra Optia® Apheresis System (Caridian BCT vers.5) when the patient's hematocrit (hct) was less than 26%. Traditional RBC exchange was modified by a depletion phase to improve efficacy. During this phase (= isovolemic hemodilution) HbS-containing red cells were removed without admixture of donor red cells by using plasma as replacement fluid. To prevent adverse side effects caused by adenine, the additive solution in the RBC concentrates was replaced by saline solution or plasma. Results: The therapeutic apheresis was feasible repeatedly via peripheral venous access (flow: 30 ± 3 mL/min, anticoagulant (AC) infusion rate 0.8 – 1.1mL/min/L total blood volume, inlet: AC ratio 13: 1) and no adverse side effects were observed. The median interval between the procedures was 42 ± 2 days. The depletion time (replacement fluid: plasma) was 25 ± 5 min, the exchange time 90 ± 10 min. The hct value at the end of the procedure was between 33 and 34%, the fraction of the patient's remaining cells (FCR) was 24 ± 2% (calculated by the device). Conclusion: RBC exchange with the Optia Spectra® System is a fast and safe method for reducing sickle cells, particularly efficient in combination with a depletion phase. Compared to blood transfusions alone, the HbS concentration declines rapidly by depleting and replacing sickle cells with normal RBCs without hyperviscosity or iron overload. However, only long-term surveillance and treatment of a larger patient collective will show whether it is possible to prevent vaso-occlusive crises and organ damage over a longer time period. Disclosures: No relevant conflicts of interest to declare.


2011 ◽  
Vol 101 (8) ◽  
pp. 1921-1929 ◽  
Author(s):  
John P. Hale ◽  
C. Peter Winlove ◽  
Peter G. Petrov

Cells ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 811
Author(s):  
Camille Boisson ◽  
Minke A. E. Rab ◽  
Elie Nader ◽  
Céline Renoux ◽  
Celeste Kanne ◽  
...  

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.


Lab on a Chip ◽  
2021 ◽  
Author(s):  
Yuncheng Man ◽  
Debnath Maji ◽  
Ran An ◽  
Sanjay Ahuja ◽  
Jane A Little ◽  
...  

Alterations in the deformability of red blood cells (RBCs), occurring in hemolytic blood disorders such as sickle cell disease (SCD), contributes to vaso-occlusion and disease pathophysiology. However, there are few...


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