scholarly journals Radioactive Sodium Chromate for the Study of Survival of Red Blood Cells

Blood ◽  
1954 ◽  
Vol 9 (12) ◽  
pp. 1155-1164 ◽  
Author(s):  
IRWIN M. WEINSTEIN ◽  
CARROLL L. SPURLING ◽  
HERMAN KLEIN ◽  
THOMAS F. NECHELES
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Sodium Chromate ◽  
Red Cell ◽  
Survival Times ◽  
Hemoglobin C ◽  
Cell Life ◽  
Erythrocyte Survival ◽  
Abnormal Hemoglobin ◽  
Radioactive Sodium

Abstract Cr51 erythrocyte survival times are reported in a group of patients with a variety of abnormal hemoglobin syndromes. Marked decreases in survival time are demonstrated in pure sickle cell anemia. Shortened survival times are reported in one case each of hemoglobin C disease and sickle cell-hemoglobin C disease with compensated hemolysis. Normal survival times are reported in sickle cell trait and hemoglobin C trait. Red cell life span as measured by the Cr51 technic agrees well with most published reports of survival times in these disorders in cases performed with the Ashby technic. The Cr51 method appears to be as useful in measuring the survival of erythrocytes containing abnormal hemoglobins as it has been shown to be in other hemolytic disorders as well as in normals. Its decided advantages are its simplicity, adaptability, and reliability.

scholarly journals 32DFP and 51Cr for Measurement of Red Cell Life Span in Abnormal Hemoglobin Syndromes

Blood ◽  
1969 ◽  
Vol 33 (2) ◽  
pp. 214-224 ◽  
Author(s):  
PAUL R. MCCURDY
Keyword(s):  
Life Span ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Cells ◽  
Red Cell ◽  
Daily Range ◽  
Hemoglobin C ◽  
Cell Life ◽  
Abnormal Hemoglobin ◽  
Elution Rate

Abstract The red cell life span was measured simultaneously using 51Cr and 32DFP in 21 patients with abnormal hemoglobin β chains and in 7 patients with normal hemoglobin. The patients included 9 with sickle cell anemia, 2 with sickle-β-thalassemia disease, 2 with sickle cell-hemoglobin C disease, 3 with homozygous hemoglobin C disease, 4 with sickle cell trait and 1 with hemoglobin C trait. The 51Cr elution rate from red cells carrying abnormal β chains was 1.2 per cent daily (range: 0.1-2.7 per cent; 4 patients had 2-component elution curves, the first of which was quite rapid (4.1-19.5 per cent daily) and could lead to significant error in the 51Cr estimate of red cell survival. The 51Cr elution rate for red cells with normal β chains was 1.3 per cent daily (range: 0.7-1.6 per cent). In the steady state, production of red cells was estimated from the 32DFP life span. Both figures varied with the disease process but erythropoiesis seldom obtained the 6-8 fold increase over normal that is considered to be the capability of normal bone marrow and hence a relative erythropoietic defect seems to be frequently present in patients with abnormal hemoglobin diseases. Two sibling pairs with sickle cell anemia were studied and were found to vary from each other as much as did the other patients with this disorder.

scholarly journals Paper Electrophoresis of Abnormal Hemoglobins and Its Clinical Applications

Blood ◽  
1954 ◽  
Vol 9 (9) ◽  
pp. 897-910 ◽  
Author(s):  
ARNO G. MOTULSKY ◽  
MILTON H. PAUL ◽  
E. L. DURRUM
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Paper Electrophoresis ◽  
Clinical Applications ◽  
Hemoglobin C ◽  
Hemoglobin Type ◽  
Cell Life ◽  
Practical Tool ◽  
Adult Hemoglobin

Abstract 1. Paper electrophoresis of abnormal hemoglobins is a simple and convenient technic for the study of the hereditary hemoglobinopathies. 2. A semiquantitative paper electrophoretic technic is described, which allows rather accurate quantitation of the various hemoglobin components by inspection alone. 3. For exact results, the more elaborate technics of elution or photoelectric scanning may be employed. The accuracy of these quantitative technics is illustrated by artificial mixture experiments. 4. The clinical applications of the method in the study of sickle cell disease and hemoglobin C abnormalities are discussed. Apart from the more common hemoglobin abnormalities (such as sickle cell trait, sickle cell anemia, C trait, sickle cell-hemoglobin C disease), a patient with 100 per cent hemoglobin C (homozygous hemoglobin C disease) and a Negro patient with sickle cell-thalassemia disease were discovered. Normal adult hemoglobin (hemoglobin A) was found in all other hereditary and acquired anemias studied. Slightly increased amounts of fetal hemoglobin were detected in cases of hereditary nonspherocytic hemolytic disease and aregenerative anemia. 5. This technic may be used for red cell life span determinations by serially following the disappearance of a certain hemoglobin type transfused into a patient with a different hemoglobin variety. Further applications of the technic are suggested. 6. The combination of the technics of paper electrophoresis and alkali denaturation offer an adequate, simple, and practical tool for diagnosis and investigation of hereditary hemoglobinopathies. 7. Identical apparatus and buffer may be used for serum protein electrophoresis.

scholarly journals Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB

Blood ◽  
1975 ◽  
Vol 45 (2) ◽  
pp. 273-279 ◽  
Author(s):  
PR McCurdy ◽  
L Mahmood ◽  
AS Sherman
Keyword(s):  
Cell Survival ◽  
Sickle Cell ◽  
Red Cells ◽  
Red Cell ◽  
Relative Ease ◽  
Hemoglobin C ◽  
Cell Life ◽  
Red Cell Survival ◽  
Beta 2 ◽  
The One

Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2–121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9 days), as it was for the one with S-O Arab. The S-O Arab subject had irreversibly sickled cells in the peripheral blood, shereas those with SC had few (less than 1/1000 red cells) or none. The S-O Arab hemolysate gelled at a hemmoglobin concentration (16.2 g/100ml) near that for sickle cell anemia hemolysates (15.9 plus or minus 1.0 g/100 ml; n equals 8) but significantly lower than that for SC hemolysates (21.6 plus or minus 1.9 g/100 ml; n equals 5). It seems likely that properties of S-C red cells other than their relative ease of sickling contribute significantly to their rate of hemolysis.

Red Cell Life Span in Sickle Cell Trait

1974 ◽  
Vol 51 (6) ◽  
pp. 339-343 ◽  
Author(s):  
Manuela M.R. Barbedo ◽  
Paul R. McCurdy
Keyword(s):  
Life Span ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Red Cell ◽  
Cell Life

scholarly journals Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB

Blood ◽  
1975 ◽  
Vol 45 (2) ◽  
pp. 273-279 ◽  
Author(s):  
PR McCurdy ◽  
L Mahmood ◽  
AS Sherman
Keyword(s):  
Cell Survival ◽  
Sickle Cell ◽  
Red Cells ◽  
Red Cell ◽  
Relative Ease ◽  
Hemoglobin C ◽  
Cell Life ◽  
Red Cell Survival ◽  
Beta 2 ◽  
The One

Abstract Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2–121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9 days), as it was for the one with S-O Arab. The S-O Arab subject had irreversibly sickled cells in the peripheral blood, shereas those with SC had few (less than 1/1000 red cells) or none. The S-O Arab hemolysate gelled at a hemmoglobin concentration (16.2 g/100ml) near that for sickle cell anemia hemolysates (15.9 plus or minus 1.0 g/100 ml; n equals 8) but significantly lower than that for SC hemolysates (21.6 plus or minus 1.9 g/100 ml; n equals 5). It seems likely that properties of S-C red cells other than their relative ease of sickling contribute significantly to their rate of hemolysis.

scholarly journals Metabolism of Heterogenic Hemoglobins

Blood ◽  
1963 ◽  
Vol 22 (3) ◽  
pp. 334-341 ◽  
Author(s):  
RICHARD D. LEVERE ◽  
HERBERT C. LICHTMAN ◽  
Joan Levine
Keyword(s):  
Pulmonary Tuberculosis ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Hemoglobin S ◽  
Active Pulmonary Tuberculosis ◽  
Hemoglobin A ◽  
Abnormal Hemoglobin

Abstract The relative rates of incorporation of Fe59 into heterogenic hemoglobins was studied in four patients with sickle cell trait. Three of the patients were free of superimposed disease, while one had active pulmonary tuberculosis. In all subjects there was a significantly greater incorporation of radioiron, per milligram of hemoglobin, into hemoglobin S than into hemoglobin A. The data indicate that in sickle cell trait the rates of synthesis of the heterogenic hemoglobins are not proportional to their circulating concentrations. Two interpretations appear possible. Since the size of the intra-marrow pool of hemoglobin S was not known, it is possible that there exists a smaller preformed pool of the abnormal hemoglobin, with the isotope making its appearance first in hemoglobin S. However, it is also possible that hemoglobin S is synthesized at a rate which is greater than that reflected by its circulating concentration. This implies that the relative concentrations of hemoglobin S and hemoglobin A vary from erythrocyte to erythrocyte, and that those cells with the greatest proportion of hemoglobin S are selectively destroyed.

HEMOGLOBIN C. REPORT OF THE HOMOZYGOUS CONDITION AND OF COMBINATIONS WITH NORMAL AND SICKLE CELL HEMOGLOBIN

PEDIATRICS ◽  
1955 ◽  
Vol 15 (2) ◽  
pp. 185-190
Author(s):  
James L. Morgan ◽  
Richard M. Bowles ◽  
Jerome S. Harris
Keyword(s):  
Sickle Cell ◽  
Clinical Picture ◽  
Definitive Diagnosis ◽  
Homozygous Condition ◽  
Hemoglobin C ◽  
Abnormal Hemoglobin

Two families showing abnormal hemoglobin types are described. One case represents the fifth reported instance homozygous C disease. The clinical picture is discussed, and the importance of electrophoresis in making a definitive diagnosis is stressed.

scholarly journals Sickle Cell Trait as a Tag for Bone Marrow Transplantation

Blood ◽  
1961 ◽  
Vol 17 (5) ◽  
pp. 610-617 ◽  
Author(s):  
DANIEL G. MILLER
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Sickle Cell ◽  
Marrow Transplantation ◽  
Sickle Cell Trait ◽  
Red Cell ◽  
Survival Curves ◽  
Cell Counts ◽  
Red Cell Survival ◽  
Good Agreement

Abstract The sickle cell trait appears to offer a reliable and replicable tag for bone marrow transplantation. Red cell survival curves using sickle cell and nonagglutinable cell counts show good agreement. The recipients exhibited no untoward effects as a result of receiving marrow from a donor with sickle cell trait.

Sickle cell trait and the risk of venous thromboembolism among blacks

Blood ◽  
2007 ◽  
Vol 110 (3) ◽  
pp. 908-912 ◽  
Author(s):  
Harland Austin ◽  
Nigel S. Key ◽  
Jane M. Benson ◽  
Cathy Lally ◽  
Nicole F. Dowling ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Sickle Cell Trait ◽  
Coagulation System ◽  
Cell Disease ◽  
Hemoglobin C ◽  
Increased Risk ◽  
S Allele

Abstract People with sickle cell disease have a chronically activated coagulation system and display hemostatic perturbations, but it is unknown whether they experience an increased risk of venous thromboembolism. We conducted a case–control study of venous thromboembolism that included 515 hospitalized black patients and 555 black controls obtained from medical clinics. All subjects were assayed for hemoglobin S and hemoglobin C genotypes. The prevalence of the S allele was 0.070 and 0.032 for case patients and controls, respectively (P < .001). The odds that a patient had sickle cell trait were approximately twice that of a control, indicating that the risk of venous thromboembolism is increased approximately 2-fold among blacks with sickle cell trait compared with those with the wild-type genotype (odds ratio = 1.8 with 95% confidence interval, 1.2-2.9). The odds ratio for pulmonary embolism and sickle cell trait was higher, 3.9 (2.2-6.9). The prevalence of sickle cell disease was also increased among case patients compared with controls. We conclude that sickle cell trait is a risk factor for venous thromboembolism and that the proportion of venous thromboembolism among blacks attributable to the mutation is approximately 7%.

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