Red blood cell membrane disorders

Tse, William T.; Lux, Samuel E.

doi:10.1111/j.1365-2141.1999.01130.x

Cited by 262 publications

(244 citation statements)

References 124 publications

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“…This relation agrees well with data showing that spherocytotic RBCs have a greater osmotic fragility (3). In spherocytosis, a patient's RBCs assume a spherical shape due to defects in RBC membrane-associated cytoskeletal proteins (4,5). However, osmotic fragility measurements on spherocytotic cells leave open the possibility that osmotic fragility is not due to cell shape alone, but to differences between the cytoskeletal makeup of spherocytotic and normal RBCs.…”

Section: Lifetime Dependence On Cell Shapesupporting

confidence: 88%

“…It has been shown that the osmotic fragility of spherocytic RBCs is higher than that of normal-shaped RBCs (3). However, spherocytic RBCs have a cytoskeletal protein makeup different from that of normal RBCs (4,5), a factor that could influence osmotic fragility independent of cell shape effects. It is therefore important to determine the relation between cell shape and alkaline hemolysis fragility (and osmotic fragility) for healthy RBCs.…”

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confidence: 99%

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Alkaline hemolysis fragility is dependent on cell shape: Results from a morphology tracker

et al. 2005

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Background: The morphometric analysis of red blood cells (RBCs) is an important area of study and has been performed previously for fixed samples. We present a novel method for the analysis of morphologic changes of live erythrocytes as a function of time. We use this method to extract information on alkaline hemolysis fragility. Many other toxins lyse cells by membrane poration, which has been studied by averaging over cell populations. However, no quantitative data are available for changes in the morphology of individual cells during membrane poration-driven hemolysis or for the relation between cell shape and fragility. Methods: Hydroxide, a porating agent, was generated in a microfluidic enclosure containing RBCs in suspension. Automatic cell recognition, tracking, and morphometric measurements were done by using a custom image analysis program. Cell area and circular shape factor (CSF) were measured over time for individual cells. Implementations were developed in MATLAB and on Kestrel, a parallel computer that affords higher speed that approaches real-time processing. Results: The average CSF went through a first period of fast increase, corresponding to the conversion of disco-

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Section: Lifetime Dependence On Cell Shapesupporting

confidence: 88%

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confidence: 99%

Alkaline hemolysis fragility is dependent on cell shape: Results from a morphology tracker

et al. 2005

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“…In the Northern European population, it is the most-common, inherited form of anemia, affecting approximately 1 in 1,000-2,500 individuals. In nearly 75 % of cases, the inheritance has an autosomal dominant pattern, and the other 25 % of cases present recessive forms and de novo mutations [1][2][3]. HS syndromes are a group of inherited disorders characterized by the presence of spherical-shaped erythrocytes on the peripheral blood smear [4].…”

Section: Introductionmentioning

confidence: 99%

Hereditary Spherocytosis: Evaluation of 68 Children

Konca

Söker

Taş

et al. 2014

Indian J Hematol Blood Transfus

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To determine the clinical and hematologic features of 68 children with hereditary spherocytosis (HS). In this retrospective study, we analyzed recorded information of 68 HS patients diagnosed between March 1997 and March 2007, including clinical manifestations at admission, gender, median age at diagnosis, family history, hematologic and biochemical data, patient management, complications, median age of splenectomy, and median follow-up time. Sixty-eight patients with HS (36 male and female) were investigated. The median age at diagnosis was 5.6 years (range 3 months to 18 years). Twenty-seven (39.7 %) had parents with consanguineous marriages, and 20 (29.4 %) had parents with first-degree consanguinity. Predominant clinical manifestations at admission were anemia in 59 patients (86.76 %), splenomegaly in 49 (72.05 %), and jaundice in 33 (48.52 %). Patients were classified as mild, moderate, or severe in 29.4, 61.7, and 8.8 % of patients, respectively. Five patients (7.3 %) underwent splenectomy. Major complications of HS were hemolytic, aplastic, and megaloblastic crises and cholelithiasis in 7 (10.2 %), 1 (1.4 %), 7 (10.2 %), and 6 (8.8 %) of patients, respectively. There were no deaths during follow-up. HS should be considered in evaluating possible diagnoses in patients with hemolytic anemia. In this study, the clinical course of patients with HS was relatively benign, with low proportions of patients having splenectomized and aplastic crises.

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“…It results from the deficiency or dysfunction of one of the red blood cell membrane proteins such as a spectrin, b spectrin, ankyrin, an anion channel protein (Band-3 protein), Protein 4.1 and Protein 4.2 (1). Diagnosis of moderate to severe forms of hereditary spherocytosis and other membrane disorders like hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis is conventionally done by a series of investigations which include peripheral smear examination, reticulocyte count, red cell indices, osmotic fragility, autohemolysis test, and acidified glycerol lysis test after exclusion of other hemolytic conditions like hemoglobinopathies, red cell enzymopathies, and autoimmune hemolytic anemia (2,3).…”

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confidence: 99%

Flow cytometric osmotic fragility—An effective screening approach for red cell membranopathies

Warang

Gupta

Kedar

et al. 2011

Cytometry Part B Clinical

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Background: Among the red cell membrane disorders, hereditary spherocytosis (HS) is one of the most common causes of inherited hemolytic anemia. The aim of this study was to compare the flow-cytometric approach for screening of red cell membrane disorders based on osmotic fragility with the eosin-5-maleimide (E5 0 M) dye test. A group of b-thalassemia heterozygotes were also studied.Methods: A red cell suspension was spiked with deionized water during acquisition and the count of residual red cells measured sequentially in real-time using flow cytometry. Fluorescence intensity of red cells stained with eosin-5-maleimide was also measured.Results: The hereditary spherocytosis (HS) group showed significantly decreased percentage residual red cells (9.31% 6 3.75%) (P 5 0.0091) whereas the b-thalassemia group showed a significant increase (93.56% 6 12.98%) (P 5 0.0008) compared to the normal control group (46.26% 6 11.33%). The cut off value of the flow cytometric osmotic fragility (FCM OF) test for red cell membrane disorders was 23.59% giving a sensitivity of 100% and specificity of 98%.Conclusions: The advantages of the FCM OF test are that it is quantitative, time effective and requires only deionized water for the measurement of osmotic fragility. It could be an effective first line screening approach for red cell membrane disorders in hematology laboratories where a flow cytometer is available.

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Red blood cell membrane disorders

Cited by 262 publications

References 124 publications

Alkaline hemolysis fragility is dependent on cell shape: Results from a morphology tracker

Alkaline hemolysis fragility is dependent on cell shape: Results from a morphology tracker

Hereditary Spherocytosis: Evaluation of 68 Children

Flow cytometric osmotic fragility—An effective screening approach for red cell membranopathies

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