scholarly journals Inside Cover: Red Blood Cells Polarize Green Laser Light Revealing Hemoglobin′s Enhanced Non-Fundamental Raman Modes (ChemPhysChem 18/2014)

ChemPhysChem ◽  
2014 ◽  
Vol 15 (18) ◽  
pp. 3882-3882 ◽  
Author(s):  
Katarzyna M. Marzec ◽  
David Perez-Guaita ◽  
Marleen de Veij ◽  
Don McNaughton ◽  
Malgorzata Baranska ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Laser Light ◽  
Green Laser ◽  
Hemoglobin S ◽  
Raman Modes

scholarly journals Red Blood Cells Polarize Green Laser Light Revealing Hemoglobin′s Enhanced Non-Fundamental Raman Modes

ChemPhysChem ◽  
2014 ◽  
Vol 15 (18) ◽  
pp. 3963-3968 ◽  
Author(s):  
Katarzyna M. Marzec ◽  
David Perez-Guaita ◽  
Marleen de Veij ◽  
Don McNaughton ◽  
Malgorzata Baranska ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Laser Light ◽  
Green Laser ◽  
Hemoglobin S ◽  
Raman Modes

Electron Microscopic Quantitation of Hemoglobin S Polymer in SS Red Blood Cells and Rheological Correlation

1989 ◽  
Vol 565 (1 Sickle Cell D) ◽  
pp. 409-412
Author(s):  
A. ANNE KAPERONIS ◽  
ROBERT G. KING ◽  
JEANNE A. SMITH ◽  
SHU CHIEN
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Electron Microscopic ◽  
Hemoglobin S

More Efficient Exchange of Sickle Red Blood Cells Can be Achieved By Exchanging the Densest Red Blood Cells

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3856-3856
Author(s):  
Suzanne Thibodeaux ◽  
Leah Irwin ◽  
Lita Jamensky ◽  
Kevin Schell ◽  
Una O'Doherty
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Blood Cells ◽  
Wild Type ◽  
Cell Disease ◽  
Hemoglobin S ◽  
And Control ◽  
Exchange Parameters

Abstract Background: In sickle cell disease, it is well established that cells containing two mutated hemoglobins (SS) are denser than cells containing wild-type hemoglobin. We asked whether we could more efficiently exchange red blood cells (RBCs) in sickle cell anemia patients by exploiting the denser property of RBCs containing hemoglobin SS compared to wild-type RBCs. Methods: To probe this question, we performed a series of experiments using the waste bags from sickle cell patients as simulated patients. We exchanged the simulated patient with one RBC volume using recently expired ABO compatible RBC units on a COBE Spectra apheresis instrument. We measured hematocrit and hemoglobin S levels in the simulated and control patient bag before and after the exchange. In the experimental scenario, we programmed the COBE Spectra to exchange the bottom half of the RBCs by indicating that the hematocrit was half of the true hematocrit (e.g. 21% when the hematocrit of the bag was 42%). For control exchanges we programmed the COBE Spectra to exchange the entire RBC volume by programing the hematocrit to be the true one (for this example 42%). Results: The percentage of hemoglobin S was more effectively diminished in our modified exchanges that targeted dense RBCs than in control exchanges. Our experimental exchanges were also more effective than expected for a 1 red blood cell volume exchange by Poisson calculation (n=5). In an optimized experiment, hemoglobin S was reduced from 23.7% to 1.3 % after exchanging 1 RBC volume using our modified approach while it was reduced from 23.2 to 5.4% using the control or routine exchange parameters. The same volume of donor RBCs (1 RBC volume) was used to exchange our experimental and control simulations. We obtained a 95% reduction of hemoglobin S in our experimental conditions and a 77% reduction in our control conditions. The instrument was programmed to compensate for RBC depletion with the modified RBC exchange. The compensation is necessary to maintain a constant hematocrit since more RBCs are present in the bottom half than top half of the centrifuged RBCs. Conclusions: It is possible to exchange sickle cell anemia patients more effectively by taking advantage of the fact that RBCs containing hemoglobin SS are denser than normal RBCs. Using waste products from sickle cell anemia exchanges provides an opportunity to safely optimize exchange parameters. This approach should allow us to: 1) achieve a higher reduction in hemoglobin S in patients, 2) achieve the previous levels of reduction using fewer donor units and/or 3) combine red cell exchange with other therapies for sickle cell disease, such as hydroxyurea, by taking advantage of the differential densities and selective depletion of red blood cells containing different levels of hemoglobin F. Disclosures No relevant conflicts of interest to declare.

scholarly journals Single Needle Option: An Alternative to Dual-Needle Access in Erythrocytapheresis in Patients with Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4284-4284
Author(s):  
Teresa Munson ◽  
Jun Zhao ◽  
Esther Elise Knapp ◽  
Charmaine Du Toit ◽  
Ashok Raj ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Vascular Access ◽  
Blood Cells ◽  
Red Cells ◽  
Cell Disease ◽  
Inlet Flow ◽  
Flow Rates ◽  
Hemoglobin S

Abstract Background: Erythrocytapheresis or red cell exchange (RCE) is an invaluable treatment option for patients with complications related sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and recurrent pain crisis. The procedure entails the removal of each patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying non-sickled hemoglobin. Adequate vascular access is essential for erythrocytapheresis to allow for high flow rates and various forms of access are used including peripheral veins and central venous access devices. Our center typically uses a single vortex port (Angiodynamics, Walnut Creek, CA) with placement of a peripheral IV at time of procedure in order to maintain a circuit for exchange. Using peripheral access reliably becomes particularly difficult in young patients and those that require multiple access over time due to scaring. To ensure a successful procedure in patients with poor peripheral access, a single-needle (SN) option for TPE (SN-TPE) that is available on Spectra Optia (Terumo BCT, Lakewood, CO) was used. The single-needle procedure utilizes intermittent, rather than continuous, flow, and thus requires extra procedure run time. One discontinuous cycle consists of "exchanging red cells," which is the drawing of blood and removal of the red cells, and "adjusting the volume in the reservoir," which is the returning of blood. These cycles continue until the procedure is complete. This procedure allows us to continue RCE in a select number of patients with poor vascular access. Methods: We evaluated our institutional experience on patients treated using single-needle RCE. In addition, information regarding each RCE session was collected including duration of procedure and inlet flow rate. Results: An average of 45 RCE procedures are performed each month. Patients are scheduled every 3 to 8 weeks, with an average of every 4-5 week frequency. We started the Single Needle option in July of 2019 on 3 patients: one adult aged patient and 2 pediatric patients. By the end of 2019 we had perform a total of 27 SN procedures. In 2020, we performed a total of 112 SN procedures, average of 9 procedures each month. As of the first 6 months of 2021, we have completed 35 SN procedures, averages 6 a month. In patients undergoing single needle exchange we were able to increase inlet flow rates from an average of 30-50ml/min to 60-80ml/min. This decreased the duration of run times from 120-198 min to 77- 119 min. Pre and post hemoglobin S% was comparable between dual and single exchange patients and there was no change in the interval between RCE sessions. Conclusion: With our increasing experience with single-needle RCE, our findings suggest that RCE can be successfully completed using the single-needle option with no impact on pre- and post-exchange hemoglobin S% levels. There was a reduction in the total length of procedure due to ability to maintain higher inlet rates and decreased time to obtain access for RCE. The single needle option also improved patient satisfaction due to more reliable access and negating need for peripheral IV access. Disclosures Munson: Terumo Medical Corporation: Consultancy, Honoraria, Speakers Bureau. Raj: Forma therapeutics: Consultancy; Terumo Medical Corporation: Honoraria, Speakers Bureau; Global biotherapeutics: Speakers Bureau.

scholarly journals Vaso-Occlusion in Sickle Cell Disease: Is Autonomic Dysregulation of the Microvasculature the Trigger?

2019 ◽  
Vol 8 (10) ◽  
pp. 1690 ◽  
Author(s):  
Saranya Veluswamy ◽  
Payal Shah ◽  
Christopher Denton ◽  
Patjanaporn Chalacheva ◽  
Michael Khoo ◽  
...  
Keyword(s):  
Blood Flow ◽  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Critical Role ◽  
Microvascular Blood Flow ◽  
Cell Disease ◽  
Hemoglobin S

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by polymerization of hemoglobin S upon deoxygenation that results in the formation of rigid sickled-shaped red blood cells that can occlude the microvasculature, which leads to sudden onsets of pain. The severity of vaso-occlusive crises (VOC) is quite variable among patients, which is not fully explained by their genetic and biological profiles. The mechanism that initiates the transition from steady state to VOC remains unknown, as is the role of clinically reported triggers such as stress, cold and pain. The rate of hemoglobin S polymerization after deoxygenation is an important determinant of vaso-occlusion. Similarly, the microvascular blood flow rate plays a critical role as fast-moving red blood cells are better able to escape the microvasculature before polymerization of deoxy-hemoglobin S causes the red cells to become rigid and lodge in small vessels. The role of the autonomic nervous system (ANS) activity in VOC initiation and propagation has been underestimated considering that the ANS is the major regulator of microvascular blood flow and that most triggers of VOC can alter the autonomic balance. Here, we will briefly review the evidence supporting the presence of ANS dysfunction in SCD, its implications in the onset of VOC, and how differences in autonomic vasoreactivity might potentially contribute to variability in VOC severity.

Study of Dense Red Blood Cells in Children with Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4870-4870
Author(s):  
Basil Coulon ◽  
Gaetana Di Liberto ◽  
Orianne Wagner Ballon ◽  
Roberto Motterlini ◽  
Xavier Decrouy ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Univariate Analysis ◽  
Organ Damage ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Distribution Method ◽  
Hemoglobin S

Abstract Many Sickle cell disease (SCD) manifestations begin at an early age and with wide inter-individual variability. High Density Red Blood Cells (DRBCs) are a specific feature of SCD, assessed with phthalate density distribution method. RBCs dehydration causes higher polymerisation of hemoglobin S and sickling, and their percentage is associated to chronic organ damage and hemolysis in adults. The spleen is one of the first altered organs, with considered complete asplenia around the age of five. We assessed the percentage of DRBCs (%DRBCs) in 50 children followed in the Créteil Paediatric Sickle Cell Referral Centre. Eligible patients had hemoglobin SS or Hemoglobin S-β0 thalassemia, without recent blood transfusion. Endpoints were the %DRBCs, its correlation with hemolytic parameters, with splenic function, with clinical manifestation. In this first prospective study in pediatric population, %DRBCs was associated with age, with particular low rate before the age of four. %DRBCs was significantly associated with enhanced haemolysis: higher total bilirubin, higher reticulocytes count, and lower hemoglobin. The results concerning LDH and ASAT were more questionable. A higher %DRBCs was associated with a lower hemoglobin F percentage. The percentage of Pitted cells, reflecting spleen function, was correlated with %DRBCs in a univariate analysis. There was no association between %DRBCs and vaso-occlusive symptoms, as expected. No statistical correlation has been found between intracranial velocity and %DRBCs. Among the five patients suffering from chronic complications related to hemolysis (kidney disease, priapism), 4 had high %DRBCs. Thus, DRBCs appearance seems to be related to the impairment of the spleen function. As in SCD adults, anemia and hemolytic markers are associated with high %DRBCs. Longitudinal analysis, with DRBC measurements before any treatment intensification, in a larger population, will be the key to evaluate the prognostic value of DRBCs for organ damage. Disclosures No relevant conflicts of interest to declare.

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