scholarly journals Intraerythrocytic Hemoglobin Crystals in Sickle Cell-Hemoglobin C Disease

Blood ◽  
1965 ◽  
Vol 25 (2) ◽  
pp. 218-223 ◽  
Author(s):  
L. W. DIGGS ◽  
ANN BELL
Keyword(s):  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Blood Corpuscle ◽  
Hemoglobin C ◽  
Blood Smears

Abstract On 70 per cent of the blood smears from 60 cases of electrophoretically proven sickle cell-hemoglobin C disease, there is observed a misshapen erythrocyte that contains condensed hemoglobin crystals which are dark-hued, homogeneous and elongated and which have parallel sides with one end terminating in a pyramid or rounded shape. A red blood corpuscle may have multiple protuberances at varying angles to each other. The incidence of intracellular hemoglobin crystals was found to be 0-24 per 1000 red blood cells with an average of 3.2/1000. Recognition of this unusual morphology is presumptive evidence of sickle cell-hemoglobin C and warrants examination by electrophoretic procedures.

scholarly journals Distribution of Abnormal Hemoglobins in Puerto Rico and Survival Studies of Red Blood Cells Using Cr51

Blood ◽  
1959 ◽  
Vol 14 (3) ◽  
pp. 255-261 ◽  
Author(s):  
RAMON M. SUAREZ ◽  
ROBERTO BUSO ◽  
LEO M. MEYER ◽  
S. T. OLAVARRIETA
Keyword(s):  
Puerto Rico ◽  
Red Blood Cells ◽  
Puerto Rican ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Blood Cells ◽  
Half Life ◽  
Hemoglobin S ◽  
Negro Population ◽  
Hemoglobin C

Abstract 1. A survey of the incidence of abnormal hemoglobins in different racial groups distributed over the island of Puerto Rico was performed. The relation of the rate of destruction of red cells to the presence of abnormal hemoglobin patterns in the affected population was also studied. 2. The abnormal hemoglobins were classified by the difference in paper electrophoretic mobility. Fetal hemoglobin was measured by its resistance to alkaline denaturation. The red cell life span was determined by measuring the survival of erythrocytes labelled with radioactive sodium chromate. 3. A total of 2,089 inhabitants were studied. There were 1,487 white subjects and 602 Negroes. Forty-two individuals were found to harbor abnormal hemoglobins. All but one were Negroes or Negroid of African descent, and their relative numbers agreed closely with the geographical distribution of ethnic groups in the island. Abnormal hemoglobins were found in 2.01 per cent of the entire series of 2,089 persons, but in those considered Negroes or Negroids the incidence of abnormal hemoglobins was 6.8 per cent. Of the 42 persons showing abnormal hemoglobins, thirty-four or 81 per cent, had hemoglobin S; only two of these had sickle cell anemia. The incidence of the sickle cell trait among the Puerto Rican Negro population was 5.2 per cent; and the incidence of hemoglobin S disease among those harboring the trait was 5.9 per cent. Eight, or 19 per cent of the abnormal cases had hemoglobin C; only one of these had hemoglobin C disease with clinical hemolytic anemia. The incidence of the hemoglobin C trait among the Puerto Rican Negro population is 1.3 per cent; and the incidence of hemoglobin C disease among those harboring the trait may reach 12.5 per cent. 4. All 42 cases harboring abnormal hemoglobins showed very small quantities of fetal or alkali-resistant hemoglobin ranging from 0.45 per cent to 3.25 per cent, averaging 1.12 per cent. 5. The "apparent" half-life of the red blood cells was found to be 10 days in sickle cell anemia, 7 days in SC disease, 20.6 days in cases of hemoglobin SA, 18.5 days in one case of hemoglobin C disease and 21.5 days in 2 cases of the combination CA. The normal "apparent" half-life in our laboratory is 24.5 days.

Hemoglobin crystals of the red blood cells in the human renal cortex: electron microscopic observations of the renal lithiasis

1978 ◽  
Vol 36 (2) ◽  
pp. 480-481
Author(s):  
Kosuke Ueda ◽  
Hiroto Washida ◽  
Nakazo Watari
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Renal Cortex ◽  
Uranyl Acetate ◽  
Osmic Acid ◽  
Renal Lithiasis ◽  
Electron Microscopic ◽  
Hemoglobin C ◽  
First Case ◽  
Ultrathin Sections

IntroductionHemoglobin crystals in the red blood cells were electronmicroscopically reported by Fawcett in the cat myocardium. In the human, Lessin revealed crystal-containing cells in the periphral blood of hemoglobin C disease patients. We found the hemoglobin crystals and its agglutination in the erythrocytes in the renal cortex of the human renal lithiasis, and these patients had no hematological abnormalities or other diseases out of the renal lithiasis. Hemoglobin crystals in the human erythrocytes were confirmed to be the first case in the kidney.Material and MethodsTen cases of the human renal biopsies were performed on the operations of the seven pyelolithotomies and three ureterolithotomies. The each specimens were primarily fixed in cacodylate buffered 3. 0% glutaraldehyde and post fixed in osmic acid, dehydrated in graded concentrations of ethanol, and then embedded in Epon 812. Ultrathin sections, cut on LKB microtome, were doubly stained with uranyl acetate and lead citrate.

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

1996 ◽  
Vol 76 (03) ◽  
pp. 322-327 ◽  
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.

Microfluidic electrical impedance assessment of red blood cell mediated microvascular occlusion

Lab on a Chip ◽  
2021 ◽  
Author(s):  
Yuncheng Man ◽  
Debnath Maji ◽  
Ran An ◽  
Sanjay Ahuja ◽  
Jane A Little ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Blood Cells ◽  
Electrical Impedance ◽  
Cell Disease ◽  
Blood Disorders

Alterations in the deformability of red blood cells (RBCs), occurring in hemolytic blood disorders such as sickle cell disease (SCD), contributes to vaso-occlusion and disease pathophysiology. However, there are few...

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

Hematology ◽  
2007 ◽  
Vol 2007 (1) ◽  
pp. 84-90 ◽  
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.

scholarly journals Role of Calcium in Phosphatidylserine Externalisation in Red Blood Cells from Sickle Cell Patients

Anemia ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-8 ◽  
Author(s):  
Erwin Weiss ◽  
David Charles Rees ◽  
John Stanley Gibson
Keyword(s):  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Phosphatidylserine Exposure ◽  
Channel Inhibition ◽  
Cation Conductance ◽  
Gardos Channel ◽  
Cation Permeability

Phosphatidylserine exposure occurs in red blood cells (RBCs) from sickle cell disease (SCD) patients and is increased by deoxygenation. The mechanisms responsible remain unclear. RBCs from SCD patients also have elevated cation permeability, and, in particular, a deoxygenation-induced cation conductance which mediates entry, providing an obvious link with phosphatidylserine exposure. The role of was investigated using FITC-labelled annexin. Results confirmed high phosphatidylserine exposure in RBCs from SCD patients increasing upon deoxygenation. When deoxygenated, phosphatidylserine exposure was further elevated as extracellular [] was increased. This effect was inhibited by dipyridamole, intracellular chelation, and Gardos channel inhibition. Phosphatidylserine exposure was reduced in high saline. levels required to elicit phosphatidylserine exposure were in the low micromolar range. Findings are consistent with entry through the deoxygenation-induced pathway (), activating the Gardos channel. [] required for phosphatidylserine scrambling are in the range achievablein vivo.

scholarly journals Babesia microti Primarily Invades Mature Erythrocytes in Mice

2006 ◽  
Vol 74 (6) ◽  
pp. 3204-3212 ◽  
Author(s):  
Ingo Borggraefe ◽  
Jie Yuan ◽  
Sam R. Telford ◽  
Sanjay Menon ◽  
Rouette Hunter ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Inbred Mouse ◽  
Flow Cytometric Analysis ◽  
Babesia Microti ◽  
Mouse Strains ◽  
Strongly Correlated ◽  
Mature Erythrocyte ◽  
Blood Smears ◽  
Nucleic Acid Stain

ABSTRACT Babesia microti is a tick-borne red blood cell parasite that causes babesiosis in people. Its most common vertebrate reservoir is the white-footed mouse. To determine whether B. microti invades reticulocytes, as does the canine pathogen B. gibsoni, we infected the susceptible inbred mouse strains C.B-17.scid and DBA/2 with a clinical isolate of B. microti. Staining of fixed permeabilized red blood cells with 4′,6′-diamidino-2-phenylindole or YOYO-1, a sensitive nucleic acid stain, revealed parasite nuclei as large bright dots. Flow cytometric analysis indicated that parasite DNA is primarily found in mature erythrocytes that expressed Babesia antigens but not the transferrin receptor CD71. In contrast, CD71-positive reticulocytes rarely contained Babesia nuclei and failed to express Babesia antigens. Accordingly, the frequency of YOYO-1-positive, CD71-negative cells strongly correlated with parasitemia, defined as the frequency of infected red blood cells assessed on Giemsa-stained blood smears. Importantly, the absolute numbers generated by the two techniques were similar. Parasitemia was modest and transient in DBA/2 mice but intense and sustained in C.B-17.scid mice. In both strains, parasitemia preceded reticulocytosis, but reticulocytes remained refractory to B. microti. In immunocompetent C.B-17 mice, reticulocytosis developed early, despite a marginal and short-lived parasitemia. Likewise, an early reticulocytosis developed in resistant BALB/cBy and B10.D2 mice. These studies establish that B. microti has a tropism for mature erythrocytes. Although reticulocytes are rarely infected, the delayed reticulocytosis in susceptible strains may result from parasite or host activities to limit renewal of the mature erythrocyte pool, thereby preventing an overwhelming parasitemia.

scholarly journals Acetylsalicylic acid and morphology of red blood cells

2010 ◽  
Vol 53 (3) ◽  
pp. 575-582 ◽  
Author(s):  
Jacques Natan Grinapel Frydman ◽  
Adenilson de Souza da Fonseca ◽  
Vanessa Câmara da Rocha ◽  
Monica Oliveira Benarroz ◽  
Gabrielle de Souza Rocha ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Control Group ◽  
Blood Samples ◽  
Morphological Alterations ◽  
Blood Smears ◽  
In Vivo Treatment ◽  
Microscopy Data

This work evaluated the effect of in vitro and in vivo treatment with ASA on the morphology of the red blood cells. Blood samples or Wistar rats were treated with ASA for one hour. Blood samples or animals treated with saline were used as control group. Blood smears were prepared, fixed, stained and the qualitative and quantitative morphology of red blood cells were evaluated under optical microscopy. Data showed that the in vitro treatment for one hour with ASA at higher dose used significantly (p<0.05) modified the perimeter/area ratio of the red blood cells. No morphological alterations were obtained with the in vivo treatment. ASA use at highest doses could interfere on shape of red blood cells.

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