scholarly journals Increased Ca++, Mg++, and Na+ + K+ ATPase activities in erythrocytes of sickle cell anemia

Blood ◽  
1982 ◽  
Vol 60 (6) ◽  
pp. 1332-1336
Author(s):  
MG Luthra ◽  
DA Sears
Keyword(s):  
Enzyme Activity ◽  
Blood Cell ◽  
Atpase Activity ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Calcium Binding ◽  
Red Cells ◽  
Low Density ◽  
Red Cell

To determine whether diminished activity of the Ca++ extrusion pump could account for the high levels of red blood cell (RBC) Ca++ in sickle cell anemia (SS), we measured calmodulin-sensitive Ca++ ATPase activity in normal and SS RBC. Hemolysates prepared with saponin were compared, since such preparations expressed maximum ATPase activities, exceeding isolated membranes or reconstituted systems of membranes plus cytosol, SS RBC hemolysates had greater Ca++ ATPase activity than normal hemolysates; they exhibited higher Mg++ and Na+ + K+ ATPase activities as well. Assays on density (age) fractions of SS and normal red cells demonstrated that all ATPase activities were highest in low density (young) cells, and activities in SS red cells exceeded those in normals in all fractions studied. Thus, when studied under conditions that maximize enzyme activity, Ca++ ATPase activity, like Mg++ and Na+ + K+ ATPase, is actually increased in SS RBC, probably due to the young red cell population present. The elevated Ca++ levels in these cells are more likely due to an increased Ca++ leak or abnormal calcium binding than to defective extrusion by the ATPase pump.

scholarly journals Increased Ca++, Mg++, and Na+ + K+ ATPase activities in erythrocytes of sickle cell anemia

Blood ◽  
1982 ◽  
Vol 60 (6) ◽  
pp. 1332-1336 ◽  
Author(s):  
MG Luthra ◽  
DA Sears
Keyword(s):  
Enzyme Activity ◽  
Blood Cell ◽  
Atpase Activity ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Calcium Binding ◽  
Red Cells ◽  
Low Density ◽  
Red Cell

Abstract To determine whether diminished activity of the Ca++ extrusion pump could account for the high levels of red blood cell (RBC) Ca++ in sickle cell anemia (SS), we measured calmodulin-sensitive Ca++ ATPase activity in normal and SS RBC. Hemolysates prepared with saponin were compared, since such preparations expressed maximum ATPase activities, exceeding isolated membranes or reconstituted systems of membranes plus cytosol, SS RBC hemolysates had greater Ca++ ATPase activity than normal hemolysates; they exhibited higher Mg++ and Na+ + K+ ATPase activities as well. Assays on density (age) fractions of SS and normal red cells demonstrated that all ATPase activities were highest in low density (young) cells, and activities in SS red cells exceeded those in normals in all fractions studied. Thus, when studied under conditions that maximize enzyme activity, Ca++ ATPase activity, like Mg++ and Na+ + K+ ATPase, is actually increased in SS RBC, probably due to the young red cell population present. The elevated Ca++ levels in these cells are more likely due to an increased Ca++ leak or abnormal calcium binding than to defective extrusion by the ATPase pump.

scholarly journals Assessment of Average Hematocrit of Red Cell Units for Erythrocytapheresis Procedure in Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4285-4285
Author(s):  
Veronica Cook ◽  
Teresa Munson ◽  
Elpidio Pena ◽  
Ashok Raj
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Small Sample ◽  
Red Cells ◽  
Red Cell ◽  
Limited Data ◽  
Cell Disease ◽  
Consensus Report

Abstract Background: Erythrocytapheresis, or red blood cell exchange (RCE) is a non-invasive procedure in which a patient's erythrocytes are removed from the bloodstream while being replaced with erythrocytes from blood donors. RCE is commonly used as a transfusion technique in patients with sickle cell disease (SCD) to help treat and prevent complications associated with sickling of erythrocytes and iron overload. The AABB consensus report (1) outlines the procedural guidelines for RCE including appropriate indications and ideal forms of vascular access. However, the guidelines make no recommendation for determining the hematocrit (Hct) of the red cell bags for the procedure. Institutions take several different approaches to determine how many red cell units to exchange. The number of units needed depends on the volume and Hct of the individual units as well as the patient's pre-procedure Hct and HbS levels, height, weight, and the desired HbS and Hct targets. Red cell units are routinely labeled with volume, but are not generally labeled with Hct. The AABB consensus report (1) states that some institutions use an estimated Hct of 56% to 57% for each unit. The report rationalizes the use of an estimated Hct for input for the RCE, by citing the approximated Hct of bags of red cells (55% to 60%) with additive solution produced from whole blood donation, based on limited data. The goal of our study was to determine the average Hct on the red cell units used for RCE. Methods: This study used a retrospective chart review to investigate the Hct of red cell units during a RCE in a calendar year. Our institution uses pre-storage leuko-reduced red blood cells units in citrate phosphate dextrose adenine (CPDA-1) and adsol preservative (AS-1), which are 21 days old or less for RCE. The average Hct of the bags of red cells infused during the procedure was determined by accessing each bag for a small sample of blood to determine the Hct. Data was excluded if the patient did not meet standard weight requirements (> 30 kg) or required additional treatment protocols including the use of washed cells or machine priming. Results: A total of 297 encounters were recorded for 30 different patients. A total of 1953 bags (approximately 517 liters of red cell units in volume) were administered. Data from the encounters were used to calculate measures of central tendency. The average calculated Hct for each encounter was 63.3%, with a median and mode Hct being 63% and 62%. The range of Hct from the bags was 54.6% to 72.9%. The average Hct of the bags ranged from 60.6% (in March) to 64.8 % (in July). Conclusions: Our study suggests a higher average Hct of transfused red cell units than stated in the AABB consensus report (1), which was based on limited data. Our findings indicate that a standardized average of 63.3%, would be appropriate for our institution. Conversely, our findings also signify that the standardized average Hct must be determined in each institution prior to their application for RCE. However, our data also reveals that it is possible to subject patients to estimates as far as 9.7% above and 9.6 % below the true average Hct of the red cell bags used. The process of determination of Hct for each of the red cell units increases the time of pre-service activities, laboratory costs, and the overall infusion center time for the patients leading to higher costs per infusion. Consequently, using a standardized average of the Hct would result in cost savings. We have therefore adopted the practice of using the standardized average of Hct of 63.3% for RCE in our patients. Reference: 1. Biller E, Zhao Y, Berg M, Boggio L, Capocelli KE, Fang DC, Koepsell S, Music-Aplenc L, Pham HP, Treml A, Weiss J, Wool G, Baron BW. Red blood cell exchange in patients with sickle cell disease-indications and management: a review and consensus report by the therapeutic apheresis subsection of the AABB. 2018 Aug;58(8):1965-1972. doi: 10.1111/trf.14806 Disclosures Munson: Terumo Medical Corporation: Consultancy, Honoraria, Speakers Bureau. Raj: Terumo Medical Corporation: Honoraria, Speakers Bureau; Global biotherapeutics: Speakers Bureau; Forma therapeutics: Consultancy.

Prevalence of Red Blood Cell Alloimmunization Among Beta-Thalassemia and Sickle Cell Anemia Patients: a Study from Saudi Arabia

2021 ◽  
Vol 67 (10/2021) ◽  
Author(s):  
Raed Felimban ◽  
Ahmed Alsharyufi ◽  
Jasem Aljehani ◽  
Ahmed Sahlool ◽  
Hamead Aljabri ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Beta Thalassemia

Alpha thalassemia protects sickle cell anemia patients from macro-albuminuria through its effects on red blood cell rheological properties

2014 ◽  
Vol 57 (1) ◽  
pp. 63-72 ◽  
Author(s):  
Yann Lamarre ◽  
Marc Romana ◽  
Nathalie Lemonne ◽  
Marie-Dominique Hardy-Dessources ◽  
Vanessa Tarer ◽  
...  
Keyword(s):  
Blood Cell ◽  
Rheological Properties ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Alpha Thalassemia

scholarly journals The Effects of Cyanate In Vitro on Red Blood Cell Metabolism and Function in Sickle Cell Anemia

1972 ◽  
Vol 51 (3) ◽  
pp. 566-574 ◽  
Author(s):  
Frank G. De Furia ◽  
Denis R. Miller ◽  
Anthony Cerami ◽  
James M. Manning
Keyword(s):  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Cell Metabolism ◽  
And Function

scholarly journals RED CELL STROMA PROTEIN RICH IN VITAMIN B12 DURING ACTIVE REGENERATION

1955 ◽  
Vol 102 (6) ◽  
pp. 725-731 ◽  
Author(s):  
G. H. Whipple ◽  
F. S. Robscheit-Robbins ◽  
W. F. Bale
Keyword(s):  
Blood Cell ◽  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Red Blood Cell ◽  
Red Cells ◽  
Cell Regeneration ◽  
Red Cell ◽  
Low Levels ◽  
Active Regeneration ◽  
Protein Rich

During active blood regeneration in anemia in dogs an increase occurs in the stroma protein of the red cells. When vitamin B12 with radioactive cobalt is given at the start of this blood regeneration one finds concentration of labeled B12 in the stroma protein but not in the hemoglobin. After the acute phase of red cell regeneration is ended the concentration of B12 in stroma protein falls rapidly to very low levels within 2 weeks. Subsequent episodes of red blood cell regeneration seems not to cause remobilization of radioactive cobalt into red cells from other body stores. It appears that the vitamin B12 is a factor of importance in the first steps of stroma protein formation in the first few days of the life of the red cell in the dog. This response in dogs and the response in pernicious anemia to vitamin B12 may have some points in common. Distribution of the B12-radioactive cobalt in the organs and tissues at autopsy has been recorded. Some very suggestive localizations were noted and some variation 1 week and 7 weeks after B12 injections. Radioactive cobalt escapes in the urine during the weeks following B12 injections.

Red Blood Cell Deformability Demonstrated with the Oxygenscan: Exploring the Association with Hydroxyurea Treatment, Co-Inherited α-Thalassemia, and Frequency of Pain in Children with Sickle Cell Anemia

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4834-4834
Author(s):  
Amina Nardo-Marino ◽  
Jesper Petersen ◽  
Andreas Glenthoej ◽  
John N. Brewin ◽  
Joergen Kurtzhals ◽  
...  
Keyword(s):  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Hemoglobin F ◽  
Positive Correlation ◽  
King's College ◽  
Hydroxyurea Treatment ◽  
Pain Frequency ◽  
Rbc Deformability

Background Sickle hemoglobin (hemoglobin S, HbS) is a structural variant of adult hemoglobin. HbS polymerizes when oxygen tensions are low, leading to red blood cell (RBC) deformation, so-called "sickling". In sickle cell anemia (SCA), loss of RBC deformability is considered to be a primary factor responsible for vaso-occlusion and hemolysis. Until recently no laboratory tests to measure RBC deformability in SCA have been readily available. Study Aims In this study we examine RBC deformability, measured with the oxygenscan module of the Laser Optical Rotational Red Cell Analyzer (Lorrca) ektacytometer, in children with SCA treated with or without hydroxyurea (HU). Furthermore, we investigate the relationship between RBC deformability and pain frequency, as well as genetic and laboratory measures known to be associated with disease severity in SCA. Methods We included children aged 0-16 years with a confirmed diagnosis of SCA (HbSS) from the pediatric sickle cell clinic at King's College Hospital in London. Children were excluded if they had received any blood transfusions within 3 months of study inclusion. Children on HU were only included if treatment had been initiated >3 months prior to recruitment and the dose was stable. Children and their parents or guardians reported frequency of pain as: daily, weekly, monthly, yearly, or never. Laboratory measurements, including total hemoglobin (hb), hemoglobin F (HbF), and reticulocyte percentage, were performed on the same day as a sample was taken for oxygenscan analysis. Data on co-inheritance of α-thalassemia was recorded if available. EDTA blood samples were kept at approximately 4°C and transported from King's College London to Copenhagen University Hospital (Herlev and Gentofte Hospital), where they were analyzed within 48 hours of sampling using the Lorrca oxygenscan (RR Mechatronics, the Netherlands). The oxygenscan measures RBC deformability expressed as an elongation index (EI) during deoxygenation and reoxygenation, with EImax expressing RBC deformability at normal oxygen concentrations, EImin expressing RBC deformability after deoxygenation, and the point of sickling (POS) expressing the point at which >5% decrease in EI is observed, representing the pO2 at which sickling begins. All statistical analyses were performed in Stata V16.0 (StataCorp. 2019, USA), using the two-sided t-test, one-way ANOVA, and Pearson's correlation when appropriate. Results We included 47 children aged 0-16 years (mean age 7.9 years) in the study, 24 (51%) receiving HU. Children in the HU group presented with significantly higher HbF percentage compared to the non-HU group (15.6% and 10.9%, p=0.03). Children receiving HU had higher EImax and EImin, and lower POS values, compared to children in the non-HU group, although results were not significant (Table 1). There was a positive correlation between HbF and EImax (r= 0.57, p=0.0001) and HbF and EImin (r= 0.56, p=0.0001), and a negative correlation between HbF and POS (r=-0.37, p=0.01), as well as a positive correlation between total hb and EImax (r=0.35, p=0.02). There was no significant correlation between any oxygenscan parameters and reticulocyte percentage. Data on α-thalassemia was available for 23 children. EImax and EImin values were higher in heterozygous children compared to children without co-inherited α-thalassemia, and POS values were lower, but results were not significant (Table 2). We found no significant association between any oxygenscan parameters and pain frequency (Table 3). Conclusion In this study we identified a strong correlation between all oxygenscan parameters and HbF percentage, as has been reported previously. We found higher EImax and EImin and lower POS values in children receiving HU treatment and children with co-inherited heterozygous α-thalassemia, suggesting increased RBC deformability in these children. These results were not significant, however, which may in part be due to lack of power in the study. Also, it is possible that children in the HU group would have presented with lower EImax and EImin and higher POS values prior to HU initiation, with treatment response leading to results similar to those found in the non-HU group. Finally, our results suggest that there is no association between oxygenscan parameters and self-reported frequency of pain in children with SCA. Disclosures No relevant conflicts of interest to declare.

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