scholarly journals Flow Cytometric Analysis of Erythrocytes Osmotic Fragility in Hereditary Spherocytosis: A Case‐Controlled Study Evaluating the Best Anticoagulant, Sample Pre‐Treatment and NaCl Concentration for Reliable Screening of this Red Blood Cell Membrane Disorder

2018 ◽  
Vol 94 (6) ◽  
pp. 910-917
Author(s):  
Camila Santos Nobre ◽  
Jeovania Almeida Silva ◽  
Rafael Henriques Jácomo ◽  
Lídia Freire Abdalla Nery ◽  
Gustavo Barcelos Barra
2014 ◽  
Vol 19 (5) ◽  
pp. 483-487 ◽  
Author(s):  
Ayako Yamamoto ◽  
Norihiro Saito ◽  
Yumiko Yamauchi ◽  
Masahide Takeda ◽  
Shigeharu Ueki ◽  
...  

2015 ◽  
Vol 135 (2) ◽  
pp. 88-93 ◽  
Author(s):  
Yi-Feng Tao ◽  
Zeng-Fu Deng ◽  
Lin Liao ◽  
Yu-Ling Qiu ◽  
Xue-Lian Deng ◽  
...  

Background: Osmotic fragility testing based on flow cytometry was recently introduced for the screening of hereditary spherocytosis (HS). This study was undertaken to evaluate the clinical diagnostic value of a flow-cytometric osmotic fragility test for HS. Methods: Peripheral blood was collected from 237 subjects at the First Affiliated Hospital of Guangxi Medical University, including 56 HS patients, 86 thalassemia patients and 95 healthy controls. The samples were examined by flow-cytometric osmotic fragility test and the percentage of residual red blood cells was used to determine HS. Peripheral blood smears were performed to examine the red blood cell morphology. Results: With clinical diagnosis of HS as the gold standard and the percentage of residual red blood cells <23.6% as the diagnostic threshold in the flow-cytometric osmotic fragility test, the sensitivity of the flow-cytometric osmotic fragility test for HS was 85.71% and the specificity was 97.24%. Conclusion: The flow-cytometric osmotic fragility test combined with a red blood cell morphology test by peripheral blood smear could be a simple, practical and accurate laboratory screening method for HS.


2020 ◽  
Vol 32 (6) ◽  
pp. 908-911
Author(s):  
Rebecca Radisic ◽  
Sean D. Owens ◽  
Charles A. Manire ◽  
Nicole Montgomery ◽  
Doug Mader ◽  
...  

Loggerhead ( Caretta caretta; Cc) and green sea ( Chelonia mydas; Cm) turtles admitted to rehabilitation facilities may require blood transfusions for supportive treatment of disorders resulting in life-threatening anemia, but, considering the unique erythrocyte chemistry of sea turtles, standardized donor red blood cell (RBC) storage protocols have not been established. Prolonged cold storage and the effects of various anticoagulant-preservative solutions have been associated with increased RBC osmotic fragility across a broad range of species. Increased RBC fragility in stored RBC products has been associated with acute transfusion reactions. The osmotic fragility test is used to measure erythrocyte resistance to hemolysis while being exposed to a series of dilutions of a saline solution. We obtained baseline measurements for osmotic fragility in healthy Cc and Cm. Osmotic fragility testing was performed on samples from 10 Cc to 10 Cm. Fifty percent (50%) RBC hemolysis was identified at a mean NaCl concentration of 0.38% in both species. Results of our study will help guide future studies evaluating optimal storage solutions for sea turtle blood products.


2001 ◽  
Vol 56 (9-10) ◽  
pp. 857-864 ◽  
Author(s):  
Federico Bordi ◽  
Cesare Cametti ◽  
Francesco De Luca ◽  
Tommaso Gili ◽  
Roberta Misasi ◽  
...  

AbstractAlterations in the electrical passive parameters of red blood cell membranes occurring during storage have been investigated by means of two different experimental approaches, i.e., radiowave dielectric spectroscopy measurements and flow-cytometric measurements. We observed a correlation between the appearance of phosphatidylserine molecules in the outer leaflet of the cell membrane and the occurrence of a change in the electrical passive membrane parameters. The electrical re-organization of the membrane, resulting in an increase of its conductivity and permittivity after 5 -7 days from blood storage, can be considered as a precursory event for the loss of asymmetry in the lipid distribution across red blood cell membrane.


2021 ◽  
Vol 12 ◽  
Author(s):  
M. C. Berrevoets ◽  
J. Bos ◽  
R. Huisjes ◽  
T. H. Merkx ◽  
B. A. van Oirschot ◽  
...  

Hereditary spherocytosis (HS) is the most common form of hereditary chronic hemolytic anemia. It is caused by mutations in red blood cell (RBC) membrane and cytoskeletal proteins, which compromise membrane integrity, leading to vesiculation. Eventually, this leads to entrapment of poorly deformable spherocytes in the spleen. Splenectomy is a procedure often performed in HS. The clinical benefit results from removing the primary site of destruction, thereby improving RBC survival. But whether changes in RBC properties contribute to the clinical benefit of splenectomy is unknown. In this study we used ektacytometry to investigate the longitudinal effects of splenectomy on RBC properties in five well-characterized HS patients at four different time points and in a case-control cohort of 26 HS patients. Osmotic gradient ektacytometry showed that splenectomy resulted in improved intracellular viscosity (hydration state) whereas total surface area and surface-to-volume ratio remained essentially unchanged. The cell membrane stability test (CMST), which assesses the in vitro response to shear stress, showed that after splenectomy, HS RBCs had partly regained the ability to shed membrane, a property of healthy RBCs, which was confirmed in the case-control cohort. In particular the CMST holds promise as a novel biomarker in HS that reflects RBC membrane health and may be used to asses treatment response in HS.


2017 ◽  
Vol 40 (1) ◽  
pp. 94-102 ◽  
Author(s):  
E. Llaudet-Planas ◽  
J. L. Vives-Corrons ◽  
V. Rizzuto ◽  
P. Gómez-Ramírez ◽  
J. Sevilla Navarro ◽  
...  

Blood ◽  
1995 ◽  
Vol 85 (3) ◽  
pp. 634-640 ◽  
Author(s):  
P Jarolim ◽  
HL Rubin ◽  
V Brabec ◽  
L Chrobak ◽  
AS Zolotarev ◽  
...  

To elucidate the molecular basis of band 3 deficiency in a recently defined subset of patients with autosomal dominant hereditary spherocytosis (HS), we screened band 3 cDNA for single-strand conformation polymorphism (SSCP). In 5 of 17 (29%) unrelated HS subjects with band 3 deficiency, we detected substitutions R760W, R760Q, R808C, and R870W that were all coinherited with the HS phenotype. The involved arginines are highly conserved throughout evolution. To examine whether or not the product of the mutant allele is inserted into the membrane, we studied one HS subject who was doubly heterozygous for the R760Q mutation and the K56E (band 3sMEMPHIS) polymorphism that results in altered electrophoretic mobility of the band 3 Memphis proteolytic fragments. We detected only the band 3MEMPHIS in the erythrocyte membrane indicating that the protein product of the mutant, R760Q, band 3 allele is absent from the red blood cell membrane. These findings suggest that the R760Q substitution, and probably the other arginine subsitutions, produce band 3 deficiency either by precluding incorporation of the mutant protein into the red blood cell membrane or by leading to loss of mutant protein from differentiating erythroid precursors.


2005 ◽  
Vol 74 (5) ◽  
pp. 396-401 ◽  
Author(s):  
Paulo Roberto Moura Lima ◽  
Mariana Ozello Baratti ◽  
Maria Lucia Chiattone ◽  
Fernando Ferreira Costa ◽  
Sara Teresinha Olalla Saad

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