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 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 Studies on Abnormal Hemoglobins

Blood ◽  
1955 ◽  
Vol 10 (5) ◽  
pp. 405-415 ◽  
Author(s):  
KARL SINGER ◽  
LILY SINGER ◽  
SEYMOUR R. GOLDBERG
Keyword(s):  
Sickle Cell Disease ◽  
Genetic Control ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Negro Population ◽  
Different Types ◽  
Adult Hemoglobin

Abstract (1) Four Negro patients with mild sickle cell-thalassemia disease (heterozygous for the genes for S hemoglobin and for thalassemia) are described. In contrast to reports in the literature, some of these patients are only mildly anemic, or not anemic at all. In three, the values for MCV and MCH are decreased, but in one, all hematologic indices are normal. All four individuals show leptocytosis and elevated reticulocyte levels. (2) Hemoglobin analyses, consisting of a combination of electrophoresis and the alkali denaturation technic, demonstrate the S + A + F pattern in three, and the S + A pattern in the fourth. These patterns are considered pathognomonic for sickle cell-thalassemia disease. They may be sharply differentiated from the S + F pattern, encountered in classical (homozygous) sickle cell anemia, and from the A + S pattern found in the heterozygous sickle cell trait. The various types of hemoglobin are reported in the sequence of their quantitative representation in the hemolysate. Hemoglobin analysis is indispensable for the recognition of the different types of sickle cell disease. (3) Evidence is cited that clinically almost asymptomatic sickle cell-thalassemia disease is probably not too rare in the American Negro population. (4) The genetic aspects of the production of fetal hemoglobin are discussed. It is postulated that the production of fetal hemoglobin is also under genetic control. The genes for fetal hemoglobin are not alleles of the genes for normal adult hemoglobin and are physiologically almost completely suppressed by the latter. Pathologic genes may render this suppression incomplete.

Developmental Pattern of Splenic Dysfunction in Sickle Cell Disorders

PEDIATRICS ◽  
1985 ◽  
Vol 76 (3) ◽  
pp. 392-397
Author(s):  
Howard A. Pearson ◽  
Diane Gallagher ◽  
Robert Chilcote ◽  
Edmund Sullivan ◽  
Judith Wilimas ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Bacterial Infections ◽  
Fetal Hemoglobin ◽  
Developmental Pattern ◽  
Cross Sectional ◽  
Serial Measurement ◽  
Hemoglobin C ◽  
Sickle Cell Disorders ◽  
Sectional Analysis

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs ≥3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin Sβ° thalassemia, splenic dysfunction (≥3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin Sβ+ thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P < .007) and directly associated with age (P ≤ .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.

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%.

scholarly journals Two Cases of Sickle Cell Disease Presumably Due to the Combination of the Genes for Thalassemia and Sickle Cell Hemoglobin

Blood ◽  
1953 ◽  
Vol 8 (5) ◽  
pp. 434-443 ◽  
Author(s):  
JAMES V. NEEL ◽  
HARVEY A. ITANO ◽  
JOHN S. LAWRENCE
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Cell Type ◽  
Cell Disease ◽  
Thalassemia Minor ◽  
Biochemical Studies

Abstract A family of Greek derivation is described in which 2 out of 6 children examined exhibited a sickle cell type of anemia. The father of these children was found to have thalassemia minor and the mother the sickle cell trait. It is presumed that the anemia in the two children was due to simultaneous heterozygosity for the sickling and thalassemia genes. Biochemical studies with reference to the occurrence and amounts of normal, sickle cell, and fetal hemoglobin were carried out on the parents and the 6 children. The theoretic interpretation of the biochemical findings is discussed.

Hereditary Persistence of Fetal Hemoglobin Caused by Single Nucleotide Promoter Mutations in Sickle Cell Trait and Hb SC Disease

Hemoglobin ◽  
2015 ◽  
Vol 40 (1) ◽  
pp. 64-65 ◽  
Author(s):  
Anthony O. Akinbami ◽  
Andrew D. Campbell ◽  
Zeqiu J. Han ◽  
Hong-Yuan Luo ◽  
David H.K. Chui ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Single Nucleotide ◽  
Hereditary Persistence ◽  
Promoter Mutations

scholarly journals Sickle Cell Trait, Hemoglobin C Trait, and Invasive Pneumococcal Disease

Epidemiology ◽  
2010 ◽  
Vol 21 (3) ◽  
pp. 340-346 ◽  
Author(s):  
Katherine A. Poehling ◽  
Laney S. Light ◽  
Melissa Rhodes ◽  
Beverly M. Snively ◽  
Natasha B. Halasa ◽  
...  
Keyword(s):  
Invasive Pneumococcal Disease ◽  
Sickle Cell ◽  
Pneumococcal Disease ◽  
Sickle Cell Trait ◽  
Hemoglobin C

scholarly journals Fetal hemoglobin that binds oxygen more strongly than adult hemoglobin saves children (and sickle-cell patients where it persists longer) from heme-binding (oxygen sequestering) proteins of anaerobic bacteria (Prevotella, et al) - thereby making SARS-Cov2 benign for them

2020 ◽  
Author(s):  
Sandeep Chakraborty
Keyword(s):  
Sickle Cell ◽  
Secondary Infection ◽  
Cardiac Injury ◽  
Anaerobic Bacteria ◽  
Fetal Hemoglobin ◽  
Ground Glass Opacity ◽  
Ground Glass ◽  
Heme Binding ◽  
Sequencing Data ◽  
Adult Hemoglobin

Here, I postulate (supported by sequencing data from patients) rationale behind two of the most critical (low SpO2 [1], need for ventilators) and baffling (doesnt effect children or malaria endemic regions [2]) effects observed in SARS-Cov2 [3–6]. In short, secondary infection with anaerobic bacteria [7–9] which express heme-expressing proteins, and can degrade and utilize hemoglobin as an effective heme source [10, 11] - causing oxygen levels (which binds to heme) to go down. Also, Prevotella is known to lower lymphocyte counts [12], increase IL-6 in plasma [13–15], cause ground glass opacity in lungs [16], and associated with cardiac injury [17] - all symptoms associated with Covid19.

scholarly journals Correlation of fetal hemoglobin in different cancer patients and sickle cell anaemia: A review

2021 ◽  
Vol 12 (1) ◽  
pp. 396-400
Author(s):  
Ayesha Shahid ◽  
Jan Peerzada Fawad ullah
Keyword(s):  
Stem Cell ◽  
Growth Factor ◽  
Cell Growth ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Fetal Hemoglobin ◽  
Globin Chain ◽  
Adult Hemoglobin ◽  
Malignant Condition ◽  
Stem Cell Growth Factor

Fetal hemoglobin is the main hemoglobin during gestation period. But this globin chain is replaced and is taken over by adult hemoglobin. Sometimes this switch from fetal to adult fails to occur leading to production of fetal hemoglobin as in case of sickle cell anemia. It is also observed that fetal hemoglobin expression is also seen under malignant condition. In malignancy the spleen, liver as well as gut acquire its ability to produce fetal hemoglobin. Certain HbF cells inducing factor such as stem cell growth factor and interleukin -3 also promote HbF erythropoiesis. HbF cells are indicated as a biomarker of tumour cells implicated in many carcinomas observed by immunohisto chemical investigations.

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