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Saudi Journal of Kidney Diseases and... Sep 2022Lupus nephritis (LN) is a major risk factor for morbidity and mortality in systemic lupus erythematosus (SLE). Urinalysis has an invaluable role in the diagnosis of...
Lupus nephritis (LN) is a major risk factor for morbidity and mortality in systemic lupus erythematosus (SLE). Urinalysis has an invaluable role in the diagnosis of various renal and urological diseases. Examinations of the urinary sediment using phase contrast microscopy (PCM) may add more information to help earlier diagnoses of LN. This cross-sectional study aimed to assess the possible role of the components of urinary sediment examined using PCM in discriminating the proliferative classes of LN (III and IV ± V) from the non-proliferative classes (I, II, and V), and to detect the correlation between the components of urinary sediment and indices of both activity and chronicity found by the renal biopsy. The study was conducted on 40 SLE patients for whom a renal biopsy was indicated. Clinical, demographic, and laboratory data and the results of the histopathological renal biopsy were collected. The morning before the renal biopsy; urine samples were collected from every patient and examined by PCM. Receiver operating characteristic curves were used to detect the area under the curve to predict proliferative LN. The correlations of counts of leukocytes, erythrocytes, all dysmorphic erythrocytes, acanthocytes, and stomatocytes with the indices of activity and chronicity were significant (activity: P = 0.027, P = 0.015, P = 0.033, P = 0.040, and P <0.001*; chronicity: P = 0.035, P = 0.009, P = 0.027, P = 0.010, and P <0.001, respectively). For patients with SLE, urinary sediment examinations can suggest a renal biopsy for the histopathology of LN.
Topics: Humans; Biomarkers; Cross-Sectional Studies; Kidney; Lupus Erythematosus, Systemic; Lupus Nephritis; Urinary Sediment Analysis; Male; Female; Adult; Middle Aged
PubMed: 37955454
DOI: 10.4103/1319-2442.389422 -
Blood Apr 1986The influence of the shape of the red blood cell during stomatocyte-echinocyte transformation on its deformability was studied by microsieving through pores with...
The influence of the shape of the red blood cell during stomatocyte-echinocyte transformation on its deformability was studied by microsieving through pores with diameters of 2.6, 4.5, and 6.9 micron. A stomatocytic transformation was produced by chlorpromazine (0.02, 0.1, and 0.5 mmol/L) and an echinocytic transformation by sodium salicylate (7.5, 30, and 120 mmol/L). For spherostomatocytes, an increase in filtration resistance through 2.6 and 4.5 micron pores was observed, whereas for spheroechinocytes, a decrease in filtration resistance through 2.6 micron pores was found. Larger pores (6.9 micron) were not sensitive to those shape changes. The changes in deformability can be explained by the fact that the surface area of (sphero)-stomatocytes decreased, whereas that of (sphero)-echinocytes increased; the cell volume remained essentially constant. Echinocytes produced by 24-hour adenosine triphosphate depletion differed from drug-induced echinocytes: they had an increased cell volume at constant surface area and consequently an increased filtration resistance through 2.6- and 4.5-micron filter pores. Shape changes with spicule formation are therefore not a homogeneous entity, and cell geometric factors (eg, surface area and volume) must be assessed with care. The viscosity of red cell suspensions at a hematocrit level of 45% was higher for drug-induced echinocytes than discocytes or stomatocytes at all shear rates tested. We conclude that the normal discocyte represents an optimum shape for the flow in vivo since a stomatocytic transformation could impair the passage through the microcirculation (decrease in cell filterability) and an echinocytic transformation could impair the flow in larger vessels (increase in blood viscosity).
Topics: Acanthocytes; Elliptocytosis, Hereditary; Erythrocyte Count; Erythrocyte Deformability; Erythrocyte Indices; Erythrocytes, Abnormal; Humans; Models, Biological; Rheology; Sodium Salicylate; Spherocytosis, Hereditary; Ultrafiltration
PubMed: 3955230
DOI: No ID Found -
British Journal of Haematology Jun 2001We describe two families with the 'cryohydrocytosis' form of stomatocytosis. Both show a mild stomatocytic anaemia with Hb levels of 12-16 g/dl and reticulocyte counts... (Review)
Review
We describe two families with the 'cryohydrocytosis' form of stomatocytosis. Both show a mild stomatocytic anaemia with Hb levels of 12-16 g/dl and reticulocyte counts of 4.3-24%, with very marked autohaemolysis at refrigerator temperatures and pseudohyperkalaemia as a result of loss of K from red cells on storage at room temperature. The ouabain + bumetanide-insensitive 'passive leak' K influx showed a 'U'-shaped temperature dependence, with a minimum at 23 degrees C. In one family, there was consistent variation in haematological severity within the pedigree. In the other, the parents of the proposita were normal, but all three of her children were affected, consistent with a new mutation of a dominant condition. Cold storage of the red cells led to a very marked increase in osmotic fragility and macrospherocytosis, explaining why a diagnosis of 'hereditary spherocytosis' can easily be reached in these pedigrees.
Topics: Anemia, Hemolytic, Congenital Nonspherocytic; Bumetanide; Cold Temperature; England; Erythrocytes; Female; Hemolysis; Humans; Lithium; Male; Osmotic Fragility; Ouabain; Pedigree; Potassium; Potassium Channels; Pregnancy; Splenectomy
PubMed: 11442486
DOI: 10.1046/j.1365-2141.2001.02792.x -
Italian Journal of Pediatrics Jul 2020Dehydrated hereditary stomatocytosis (DHS) or hereditary xerocytosis is a rare, autosomal dominant hemolytic anemia characterized by macrocytosis, presence of... (Review)
Review
BACKGROUND
Dehydrated hereditary stomatocytosis (DHS) or hereditary xerocytosis is a rare, autosomal dominant hemolytic anemia characterized by macrocytosis, presence of stomatocytes and dehydration of red blood cells (RBCs). The dehydration is caused by a defect in cellular cation content. The most frequent expression of the pathology is hemolytic well-compensated anemia with high reticulocyte count, a tendency to macrocytosis, increased mean corpuscular hemoglobin concentration (MCHC) and mild jaundice. We here describe a new mutation of PIEZO1 gene, the most frequent mutated gene in DHS, in a family affected by hereditary hemolytic anemia.
CASE PRESENTATION
We describe the case of a 12-years-old girl with well-compensated chronic hemolysis, increased MCHC and a father who had the same hematological characteristics. After excluding secondary causes of chronic hemolysis and enzymatic defects of the RBCs, microscopic observation of the peripheral blood smear, tests of RBC lysis, ektacytometry, SDS-PAGE and in last instance genetic analysis has been performed. This complex diagnostic workup identified a new variant in the PIEZO1 gene, never described in literature, causative of DHS. This pathogenetic variant was also detected in the father.
CONCLUSIONS
This case report highlights the importance of a correct and exhaustive diagnostic-workup in patients with clinical suspicious for hemolytic anemia in order to make a differential diagnosis. This is relevant for the management of these patients because splenectomy is contraindicated in DHS due to high thrombotic risk.
Topics: Adult; Anemia, Hemolytic, Congenital; Child; Female; Humans; Hydrops Fetalis; Ion Channels; Male; Mutation, Missense; Pedigree
PubMed: 32703298
DOI: 10.1186/s13052-020-00864-x -
Journal of Biomedical Optics Jan 2015Counting morphologically normal cells in human red blood cells (RBCs) is extremely beneficial in the health care field. We propose a three-dimensional (3-D)...
Counting morphologically normal cells in human red blood cells (RBCs) is extremely beneficial in the health care field. We propose a three-dimensional (3-D) classification method of automatically determining the morphologically normal RBCs in the phase image of multiple human RBCs that are obtained by off-axis digital holographic microscopy (DHM). The RBC holograms are first recorded by DHM, and then the phase images of multiple RBCs are reconstructed by a computational numerical algorithm. To design the classifier, the three typical RBC shapes, which are stomatocyte, discocyte, and echinocyte, are used for training and testing. Nonmain or abnormal RBC shapes different from the three normal shapes are defined as the fourth category. Ten features, including projected surface area, average phase value, mean corpuscular hemoglobin, perimeter, mean corpuscular hemoglobin surface density, circularity, mean phase of center part, sphericity coefficient, elongation, and pallor, are extracted from each RBC after segmenting the reconstructed phase images by using a watershed transform algorithm. Moreover, four additional properties, such as projected surface area, perimeter, average phase value, and elongation, are measured from the inner part of each cell, which can give significant information beyond the previous 10 features for the separation of the RBC groups; these are verified in the experiment by the statistical method of Hotelling's T-quare test. We also apply the principal component analysis algorithm to reduce the dimension number of variables and establish the Gaussian mixture densities using the projected data with the first eight principal components. Consequently, the Gaussian mixtures are used to design the discriminant functions based on Bayesian decision theory. To improve the performance of the Bayes classifier and the accuracy of estimation of its error rate, the leaving-one-out technique is applied. Experimental results show that the proposed method can yield good results for calculating the percentage of each typical normal RBC shape in a reconstructed phase image of multiple RBCs that will be favorable to the analysis of RBC-related diseases. In addition, we show that the discrimination performance for the counting of normal shapes of RBCs can be improved by using 3-D features of an RBC.
Topics: Algorithms; Bayes Theorem; Erythrocytes; Holography; Humans; Image Cytometry; Microscopy
PubMed: 25567613
DOI: 10.1117/1.JBO.20.1.016005 -
Journal of Biomedical Optics Jul 2017We present unsupervised clustering methods for automatic grouping of human red blood cells (RBCs) extracted from RBC quantitative phase images obtained by digital...
We present unsupervised clustering methods for automatic grouping of human red blood cells (RBCs) extracted from RBC quantitative phase images obtained by digital holographic microscopy into three RBC clusters with regular shapes, including biconcave, stomatocyte, and sphero-echinocyte. We select some good features related to the RBC profile and morphology, such as RBC average thickness, sphericity coefficient, and mean corpuscular volume, and clustering methods, including density-based spatial clustering applications with noise, k-medoids, and k-means, are applied to the set of morphological features. The clustering results of RBCs using a set of three-dimensional features are compared against a set of two-dimensional features. Our experimental results indicate that by utilizing the introduced set of features, two groups of biconcave RBCs and old RBCs (suffering from the sphero-echinocyte process) can be perfectly clustered. In addition, by increasing the number of clusters, the three RBC types can be effectively clustered in an automated unsupervised manner with high accuracy. The performance evaluation of the clustering techniques reveals that they can assist hematologists in further diagnosis.
Topics: Cell Shape; Cluster Analysis; Erythrocyte Count; Erythrocytes; Hematology; Holography; Humans
PubMed: 28742920
DOI: 10.1117/1.JBO.22.7.076015 -
Transfusion Medicine and Hemotherapy :... Feb 2021Red blood cells (RBCs) stored for transfusions can lyse over the course of the storage period. The lysis is traditionally assumed to occur via the formation of...
BACKGROUND
Red blood cells (RBCs) stored for transfusions can lyse over the course of the storage period. The lysis is traditionally assumed to occur via the formation of spiculated echinocyte forms, so that cells that appear smoother are assumed to have better storage quality. We investigate this hypothesis by comparing the morphological distribution to the hemolysis for samples from different donors.
METHODS
Red cell concentrates were obtained from a regional blood bank quality control laboratory. Out of 636 units processed by the laboratory, we obtained 26 high hemolysis units and 24 low hemolysis units for assessment of RBC morphology. The association between the morphology and the hemolysis was tested with the Wilcoxon-Mann-Whitney U test.
RESULTS
Samples with high stomatocyte counts ( = 0.0012) were associated with increased hemolysis, implying that cells can lyse via the formation of stomatocytes.
CONCLUSION
RBCs can lyse without significant echinocyte formation. Lower degrees of spiculation are not a good indicator of low hemolysis when RBCs from different donors are compared.
PubMed: 33708051
DOI: 10.1159/000508711 -
Blood Sep 1975Cation permeability and lipid composition have been studied in the red cells of five patients with various features of the hereditary stomatocytosis syndrome. Hemolysis...
Cation permeability and lipid composition have been studied in the red cells of five patients with various features of the hereditary stomatocytosis syndrome. Hemolysis was compensated in four patients, and only one patient was anemic. Cell NA+ was increased an average of 3 mueq per ml cells and cell K+ decreased 14 mueq per ml cells. Both active and passive fluxes of Na+ and K+ were increased by two to six times normal. Tritiated ouabain binding was increased an average of 2.5-fold, suggesting a proportionally greater number of cation pumps per cell. The coupling ratio of active Na+:K+ fluxes was normal (3:2). Calcium permeability was increased compatible with the degree of reticulocytosis, and cell Ca2+ content was normal. The lowered sum of Na+ plus K+ was associated with a high MCHC and low cell water. When examined in wet preparations, red cells assumed either a bowl-shaped or an irregular contour, and they appeared as target cells on dry smears. Only when cell water was increased in hypotonic media were stomatocytes seen on smear. The total lipid content of red cells was increased in four patients, although it was normal in one. The mole ratio of cholesterol to phospholipid was always normal; however, phospholipid analysis showed an increased proportion of phosphatidyl choline. The abnormal cells were osmotically resistant due to both an increased membrane surface area and a low total cation content. These patients show two hallmarks of hereditary stomatocytosis: bowlshaped red cells observed on wet preparations and a marked increase in Na+ and K+ permeability. The heterogeneity of this syndrome in our patients and in others reported with hereditary stomatocytosis appears to result from (1) variability in the increase in surface area which results from an excess of membrane lipid content, particularly phosphatidylcholine, and (2) a variability in cell water content which may be either decreased or increased as a result of changes in the sum of Na+ plus K+ ions.
Topics: Anemia, Hemolytic; Calcium; Carbon Radioisotopes; Cell Membrane; Cell Membrane Permeability; Child; Erythrocyte Count; Erythrocytes, Abnormal; Female; Hematocrit; Hemoglobins; Humans; Hypotonic Solutions; Lipids; Male; Middle Aged; Osmotic Fragility; Ouabain; Potassium; Reticulocytes; Sodium; Sodium Radioisotopes; Sucrose; Tritium; Water-Electrolyte Balance
PubMed: 1148392
DOI: No ID Found