scholarly journals The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

Blood ◽  
1988 ◽  
Vol 72 (6) ◽  
pp. 2056-2059 ◽  
Author(s):  
WM Lande ◽  
DL Andrews ◽  
MR Clark ◽  
NV Braham ◽  
DM Black ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Hemoglobin Concentration ◽  
Red Cells ◽  
Strong Positive Correlation ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Cellular Dehydration ◽  
Painful Crisis

Abstract To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.

scholarly journals The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

Blood ◽  
1988 ◽  
Vol 72 (6) ◽  
pp. 2056-2059
Author(s):  
WM Lande ◽  
DL Andrews ◽  
MR Clark ◽  
NV Braham ◽  
DM Black ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Hemoglobin Concentration ◽  
Red Cells ◽  
Strong Positive Correlation ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Cellular Dehydration ◽  
Painful Crisis

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.

Roles of cell geometry and cellular viscosity in red cell passage through narrow pores

1985 ◽  
Vol 248 (5) ◽  
pp. C473-C479 ◽  
Author(s):  
W. H. Reinhart ◽  
S. Chien
Keyword(s):  
Pore Size ◽  
Hemoglobin Concentration ◽  
Red Cells ◽  
Mean Corpuscular Hemoglobin ◽  
Red Cell ◽  
Cell Passage ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Narrow Channels ◽  
The Mean

The relative roles of two fundamental determinants of red cell deformability, namely cell size and cellular viscosity, in affecting red cell passage through narrow channels have been assessed by determining the filterability of red cells subjected to osmotic variations. Suspensions of red cells (10(6) cells/microliter) in eight different osmolalities ranging from 172 +/- 3 (mean +/- SD) to 665 +/- 28 mosmol/kg H2O were filtered through polycarbonate sieves with three different pore diameters (2.6 +/- 0.2, 4.5 +/- 0.6, and 6.9 +/- 0.8 micron). The mean corpuscular volume varied inversely with osmolality and ranged from 149 +/- 9 to 67 +/- 10 fl; the mean corpuscular hemoglobin concentration varied directly with osmolality and ranged from 23.7 +/- 0.8 to 55.9 +/- 3.9 g/dl. The filtration data were analyzed with a theoretical model to derive the parameter beta, which is the ratio of resistance in a pore bearing a red blood cell to that in a pore filled with the suspending medium alone. For each pore size, beta showed a V-shaped relationship with osmolality; the optimum osmolality for minimum beta varied inversely with the pore size. For the small 2.6-micron pores, the minimum beta was attained following hyperosmotic shrinkage of the red cells at 400 mosmol/kg H2O, whereas passage through the large 6.9-micron pores was facilitated by hypoosmotic swelling of the red cells in about 200 mosmol/kg H2O. Red cell filtration through small pores is more sensitive to alterations in cell volume, whereas that through large pores is primarily determined by changes in cellular viscosity.(ABSTRACT TRUNCATED AT 250 WORDS)

The Mechanism of Hemolysis in Sickle Cell Anemia.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1439-1439
Author(s):  
Tennille Presley ◽  
Lauren Bain ◽  
Samir Ballas ◽  
James Nichols ◽  
Hernan Sabio ◽  
...  
Keyword(s):  
Oxidative Damage ◽  
Sickle Cell ◽  
Large Animal ◽  
Red Cell ◽  
Intravascular Hemolysis ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Aerobic Conditions ◽  
Sickle Hemoglobin

Abstract Accumulating transgenic animal, large animal and human epidemiological evidence supports a role for hemolysis in the pathobiology of sickle cell disease. However, the mechanism of hemolysis or more specifically the relative contribution of sickling and oxidative damage has yet to be determined. Early studies have shown that repetitive sickling/unsickling via cycles of deoxygenation/reoxygenation lead to a decrease in sickle red cell deformability (even under oxygenated conditions), suggesting an important role for sickle hemoglobin polymerization probably associated with membrane loss and dehydration. However, all of these previous studies have used sickle (SS) cells which undergo cycles of sickling and unsickling in vivo and hence, have poor deformability even under aerobic conditions prior to in vitro experiments. In our study, we used sickle cell trait cells (AS) which do not sickle under physiological oxygen pressures, but can be sickled by exposing them to anoxia (zero percent oxygen). This novel approach allows us to study the effects of sickle hemoglobin polymerization on cells that have never contained polymers before, in order to gain information on the role of polymerization in intravascular hemolysis. We measured deformability in normal (AA), AS, and SS red cells using flow channel laser diffraction and obtained a deformability coefficient (the lower the coefficient the poorer the deformability). In addition, we measured mechanical fragility via shaking in the presence of glass beads followed by measurements of plasma hemoglobin using absorption spectroscopy. As expected, there was no difference in deformability measured for AA cells under aerobic or anaerobic conditions (2.1 ± 0.5 oxy vs. 1.9 ± 0.4 deoxy, n=3), while the deformability of deoxygenated SS or AS cells was substantially decreased, indicating that polymers formed for both SS and AS cells (1.6 ± 0.3 oxy SS vs. 1.34 ± 0.05 deoxy SS; 1.8 ± 0.4 oxy AS vs. 1.17 ± 0.04 deoxy AS, n=3). Likewise, whereas partial pressure of oxygen had no significant effect on the mechanical fragility of AA cells (2.1 ± 0.3 μM for oxy vs. 1.5 ± 0.9 μM for deoxy, n=3); deoxygenation greatly increased the mechanical fragility of both AS and SS cells (1.8 ± 0.2 μM oxy AS vs. 10.6 ± 3.2 μM deoxy AS; 0.8 ± 0.1 μM oxy SS vs. 2.7 ± 0.9 μM deoxy SS). Reoxygenation of SS cells following prolonged deoxygenation, tended to not regain the level of mechanical fragility of cells maintained in continuous aerobic conditions (1.9 ± 0.4 μM reoxy vs. 0.6 ± 0.1 μM oxy); consistent with previous findings that repeated sickling and unsickling leads to diminished red cell deformability. On the other hand, AS cells fully regained their lower mechanical fragility following reoxygenation after prolonged deoxygenation (1.0 ± 0.2 μM reoxy vs. 1.1 ± 0.4 μM oxy). Our data support two important conclusions: The observed poor rheology of SS cells under aerobic conditions does not result from a single or prolonged sickling event, but rather is likely to include contributions from oxidative damage. This conclusion is based on the observation that rheological properties of deoxygenated AS cells return to normal following reoxygenation. A substantial amount of intravascular hemolysis occurs in vivo in cells that contain sickle cell hemoglobin polymers. This is suggested by the dramatic increase in mechanical fragility upon deoxygenation of both AS and SS cells. AS cells at zero oxygen pressure are likely to contain similar amounts of polymers as SS cells under physiological conditions. Thus, it is likely that many cells that hemolyze in vivo do so upon the first sickling event.

Higher fetal hemoglobin concentration in patients with sickle cell disease in eastern India reduces frequency of painful crisis

2009 ◽  
Vol 83 (4) ◽  
pp. 383-384 ◽  
Author(s):  
Ranjeet Singh Mashon ◽  
Preetinanda Manaswini Dash ◽  
Janet Khalkho ◽  
Laxmikanta Dash ◽  
Pradeep Kumar Mohanty ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Hemoglobin Concentration ◽  
Eastern India ◽  
Cell Disease ◽  
Painful Crisis

Measurements of red cell deformability and hydration reflect HbF and HbA 2 in blood from patients with sickle cell anemia

2017 ◽  
Vol 65 ◽  
pp. 41-50 ◽  
Author(s):  
Nermi L. Parrow ◽  
Hongbin Tu ◽  
James Nichols ◽  
Pierre-Christian Violet ◽  
Corinne A. Pittman ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Cell ◽  
Cell Deformability ◽  
Red Cell Deformability

Red Cell Properties In Haemostasis

1981 ◽  
Author(s):  
G M Housley ◽  
G V R Born
Keyword(s):  
Red Cells ◽  
Red Cell ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Initial Flow ◽  
Red Cell Membranes ◽  
New Hypothesis ◽  
Platelets Function

Earlier observations of ours have suggested that, under in vitro conditions resembling those under which platelets function haemostatically in vivo, their activation is promoted by the red cells. Seme of the evidence suggested that this is through limited haemolysis with release of ADP. However, newly determined time relationships make this uncertain. Could red cells provide ADP without haemolysis?Crtheir flow properties affect the process more? To analyse the problem, we are determining dependence of red cell deformability on membrane constitution; and release of haemoglobin and adeninenucleotides under different conditions. Ten percent human red cell suspensions in physiological salines flow under constant pressures through 2, 3, 4 and 5 pm micropore filters, the flow rate measured continuously with an electronic balance. Initial flow rates are increased by fluidising agents, eg. ethanol, and decreased by agents with opposite effect. Our results are consistent with the new hypothesis of S.J. Singer on the mode of action of amphipathic agents, such as chlorpromazine, on red cell membranes.

Phenotype Matching of Donor Red Blood Cell Units for Nonalloimmunized Sickle Cell Disease Patients: A Survey of 1182 North American Laboratories

2005 ◽  
Vol 129 (2) ◽  
pp. 190-193 ◽  
Author(s):  
Melanie Osby ◽  
Ira A. Shulman
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
North American ◽  
Red Cells ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Antigen ◽  
Red Cell Antigens ◽  
Matched Donor

Abstract Context.—The transfusion of donor red blood cell units (RBCs) that lack certain red cell antigens (such as C, E, and K) when the corresponding antigens are absent from the recipient's red cells has been shown to reduce the risk of red cell alloimmunization in sickle cell disease patients. However, data are limited regarding the extent to which transfusion services routinely perform red cell antigen phenotype testing of nonalloimmunized sickle cell disease patients, and then use that information to select donor RBCs lacking 1 or more of the red cell antigens that the patient's red cells do not express. Objective.—To determine the extent to which transfusion services routinely perform red cell antigen phenotype testing of nonalloimmunized sickle cell disease patients, and then use that information to select donor RBCs lacking 1 or more of the red cell antigens that the patient's red cells do not express. Design.—An educational subsection of a College of American Pathologists Proficiency Testing Survey (J-C 2003) assessed transfusion service practices regarding performance of red cell antigen phenotype testing of nonalloimmunized sickle cell disease patients and how transfusion services use this information for the selection of donor RBCs. The data analysis of the survey included 1182 North American laboratories. Results.—Data from 1182 laboratories were included in the survey analysis, of which the majority (n = 743) reported that they did not routinely perform phenotype testing of sickle cell disease patients for antigens other than ABO and D. The other 439 laboratories reported that they did routinely perform phenotype testing of sickle cell disease patients for antigens in addition to ABO and D. The majority of these 439 laboratories (three fourths; n = 330) reported that they used these patient data for prophylactic matching with donor RBCs when sickle cell disease patients required transfusion. When phenotype-matched donor RBCs were used, the antigens most commonly matched (85% of the time) were C, E, and K. Conclusions.—The majority of North American hospital transfusion service laboratories do not determine the red cell antigen phenotype of nonalloimmunized sickle cell disease patients beyond ABO and D. Those laboratories that do determine the red cell phenotype of nonalloimmunized sickle cell disease patients beyond ABO and D most commonly match for C, E, and K antigens when phenotype-matched donor RBCs are used.

Microvascular abnormalities in sickle cell disease: a computer-assisted intravital microscopy study

Blood ◽  
2002 ◽  
Vol 99 (11) ◽  
pp. 3999-4005 ◽  
Author(s):  
Anthony T. W. Cheung ◽  
Peter C. Y. Chen ◽  
Edward C. Larkin ◽  
Patricia L. Duong ◽  
Sahana Ramanujam ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Intravital Microscopy ◽  
Computer Assisted ◽  
Red Cell ◽  
Cell Disease ◽  
Computer Assisted Image ◽  
Conjunctival Vessels ◽  
Painful Crisis

The conjunctival microcirculation of 18 homozygous sickle cell disease (SCD) patients during steady-state, painful crisis, and postcrisis conditions was recorded on high-resolution videotapes using intravital microscopy. Selected videotape sequences were subsequently coded, frame-captured, studied, and blindly analyzed using computer-assisted image analysis protocols. At steady-state (baseline), all SCD patients exhibited some of the following morphometric abnormalities: abnormal vessel diameter, comma signs, blood sludging, boxcar blood flow phenomenon, distended vessels, damaged vessels, hemosiderin deposits, vessel tortuosity, and microaneurysms. There was a decrease in vascularity (diminished presence of conjunctival vessels) in SCD patients compared with non-SCD controls, giving the bulbar conjunctiva a “blanched” avascular appearance in most but not all SCD patients during steady-state. Averaged steady-state red cell velocity in SCD patients was slower than in non-SCD controls. During painful crisis, a further decrease in vascularity (caused by flow stoppage in small vessels) and a 36.7% ± 5.2% decrease in large vessel (mostly venular) diameter resulted. In addition, the conjunctival red cell velocities either slowed significantly (6.6% ± 13.1%; P < .01) or were reduced to a trickle (unmeasurable) during crisis. The microvascular changes observed during crisis were transient and reverted to steady-state baseline after resolution of crisis. When combined, intravital microscopy and computer-assisted image analysis (computer-assisted intravital microscopy) represent the availability of a noninvasive tool to quantify microvascular abnormalities in vascular diseases, including sickle cell disease. The ability to identify and relocate the same conjunctival vessels for longitudinal studies uniquely underscores the applicability of this quantitative real-time technology.

scholarly journals Red Blood Cells: Tethering, Vesiculation, and Disease in Micro-Vascular Flow

Diagnostics ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 971
Author(s):  
Robert J. Asaro ◽  
Pedro Cabrales
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Myeloproliferative Neoplasms ◽  
Red Cells ◽  
Red Cell ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Vascular Flow ◽  
Wide Range ◽  
Mechanistic Basis

The red blood cell has become implicated in the progression of a range of diseases; mechanisms by which red cells are involved appear to include the transport of inflammatory species via red cell-derived vesicles. We review this role of RBCs in diseases such as diabetes mellitus, sickle cell anemia, polycythemia vera, central retinal vein occlusion, Gaucher disease, atherosclerosis, and myeloproliferative neoplasms. We propose a possibly unifying, and novel, paradigm for the inducement of RBC vesiculation during vascular flow of red cells adhered to the vascular endothelium as well as to the red pulp of the spleen. Indeed, we review the evidence for this hypothesis that links physiological conditions favoring both vesiculation and enhanced RBC adhesion and demonstrate the veracity of this hypothesis by way of a specific example occurring in splenic flow which we argue has various renderings in a wide range of vascular flows, in particular microvascular flows. We provide a mechanistic basis for membrane loss and the formation of lysed red blood cells in the spleen that may mediate their turnover. Our detailed explanation for this example also makes clear what features of red cell deformability are involved in the vesiculation process and hence require quantification and a new form of quantitative indexing.

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.

Sign in / Sign up

Export Citation Format

Share Document