Microfluidic electrical impedance assessment of red blood cell mediated microvascular occlusion

The treatment of patients with sickle cell disease frequently requires transfusion of red blood cells. Complications due to alloimmunization of red blood cells antigen remain a major risk as a post transfusion effect. The objective of this study is to determine the frequency of red cell alloimmunization in Jeddah, Saudi Arabia. A retrospective cross-section study of sickle cell disease patients at King Abdulaziz University Hospital between 2012 and 2013 was performed. Demographic characteristics and transfusion history was recorded. Blood samples were analyzed for alloimmunization using immunohematological technique. A total of 234 sickle cell patients were analyzed, of which 30 (12.8%) showed alloantibodies. A total of 43 alloantibodies were found out of which 28 belonged to Rh group, eight belonged to Kell while three belonged to MNS group. Demographic and transfusion characteristics were analyzed between alloimmunized and nonalloimmunized sickle cell disease patients. The rate of alloimmunization in Jeddah, Saudi Arabia was 12.8%. There was significant difference observed between alloantibodies detection between transfused patients compared to non-transfused patients. The consequences of red blood cell alloimmunization are highly significant and therefore immune hematological testing is highly recommended.

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Characterization and statistical modeling of glycosylation changes in sickle cell disease

Blood Advances ◽

10.1182/bloodadvances.2020003376 ◽

2021 ◽

Vol 5 (5) ◽

pp. 1463-1473

Author(s):

Heather E. Ashwood ◽

Christopher Ashwood ◽

Anna P. Schmidt ◽

Rebekah L. Gundry ◽

Karin M. Hoffmeister ◽

...

Keyword(s):

Mass Spectrometry ◽

Sickle Cell Disease ◽

Blood Cell ◽

Red Blood Cells ◽

Blood Group ◽

Sickle Cell ◽

Red Blood Cell ◽

Blood Cells ◽

Blood Group Antigens ◽

Cell Disease

AbstractSickle cell disease is an inherited genetic disorder that causes anemia, pain crises, organ infarction, and infections in 13 million people worldwide. Previous studies have revealed changes in sialic acid levels associated with red blood cell sickling and showed that stressed red blood cells bare surface-exposed clustered terminal mannose structures mediating hemolysis, but detailed glycan structures and anti-glycan antibodies in sickle cell disease remain understudied. Here, we compiled results obtained through lectin arrays, glycan arrays, and mass spectrometry to interrogate red blood cell glycoproteins and glycan-binding proteins found in the plasma of healthy individuals and patients with sickle cell disease and sickle cell trait. Lectin arrays and mass spectrometry revealed an increase in α2,6 sialylation and a decrease in α2,3 sialylation and blood group antigens displayed on red blood cells. Increased binding of proteins to immunogenic asialo and sialyl core 1, Lewis A, and Lewis Y structures was observed in plasma from patients with sickle cell disease, suggesting a heightened anti-glycan immune response. Data modeling affirmed glycan expression and plasma protein binding changes in sickle cell disease but additionally revealed further changes in ABO blood group expression. Our data provide detailed insights into glycan changes associated with sickle cell disease and refer glycans as potential therapeutic targets.

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Increased Procoagulant Activity of Red Blood Cells from Patients with Homozygous Sickle Cell Disease and β-Thalassemia

Thrombosis and Haemostasis ◽

10.1055/s-0038-1650577 ◽

1996 ◽

Vol 76 (03) ◽

pp. 322-327 ◽

Cited By ~ 73

Author(s):

Dominique Helley ◽

Amiram Eldor ◽

Robert Girot ◽

Rolande Ducrocq ◽

Marie-Claude Guillin ◽

...

Keyword(s):

Sickle Cell Disease ◽

Red Blood Cells ◽

Sickle Cell ◽

Blood Cells ◽

Annexin V ◽

Mean Value ◽

Procoagulant Activity ◽

Dependent Manner ◽

Cell Disease ◽

Homozygous Sickle Cell Disease

SummaryIt has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous β-thalassemia behave as procoagulant cells. The procoagulant activity of β-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i. e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with β-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or (3-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 µM) than in the absence of cells (26 µM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 µM) or β-thalassemia RBCs (mean value: 1.5 pM) was significantly lower compared to normal RBCs (p <0.001). No significant difference was observed between SS-RBCs and p-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and (3-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both β-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.

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Drugs for preventing red blood cell dehydration in people with sickle cell disease

10.1002/14651858.cd003426 ◽

2002 ◽

Cited By ~ 13

Author(s):

C Riddington ◽

L De Franceschi

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Cell Disease ◽

Cell Dehydration

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Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease

Cells ◽

10.3390/cells10040811 ◽

2021 ◽

Vol 10 (4) ◽

pp. 811

Author(s):

Camille Boisson ◽

Minke A. E. Rab ◽

Elie Nader ◽

Céline Renoux ◽

Celeste Kanne ◽

...

Keyword(s):

Steady State ◽

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Cell Disease ◽

Oxygen Gradient ◽

Acute Complication ◽

Adults And Children ◽

The Difference

(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.

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Role of Adhesion Molecules and Vascular Endothelium in the Pathogenesis of Sickle Cell Disease

Hematology ◽

10.1182/asheducation-2007.1.84 ◽

2007 ◽

Vol 2007 (1) ◽

pp. 84-90 ◽

Cited By ~ 27

Author(s):

Marilyn J. Telen

Keyword(s):

Sickle Cell Disease ◽

Red Blood Cells ◽

Adhesion Molecules ◽

Sickle Cell ◽

Vascular Endothelium ◽

Blood Cells ◽

Cell Disease ◽

Adhesive Interactions ◽

Lines Of Evidence

AbstractA number of lines of evidence now support the hypothesis that vaso-occlusion and several of the sequelae of sickle cell disease (SCD) arise, at least in part, from adhesive interactions of sickle red blood cells, leukocytes, and the endothelium. Both experimental and genetic evidence provide support for the importance of these interactions. It is likely that future therapies for SCD might target one or more of these interactions.

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