scholarly journals Hemoglobin F to Total Hemoglobin Ratio Measurement

2020 ◽  
Author(s):  
Keyword(s):  
Hemoglobin F ◽  
Ratio Measurement ◽  
Total Hemoglobin

scholarly journals Hb Leslie, an unstable hemoglobin due to deletion of glutaminyl residue beta 131 (H9) occurring in association with beta0-thalassemia, HbC, and HbS

Blood ◽  
1976 ◽  
Vol 47 (1) ◽  
pp. 99-112 ◽  
Author(s):  
CL Lutcher ◽  
JB Wilson ◽  
ME Gravely ◽  
PD Stevens ◽  
CJ Chen ◽  
...  
Keyword(s):  
Chemical Analysis ◽  
Electrophoretic Mobility ◽  
Clinical Manifestations ◽  
Beta Chain ◽  
Hemoglobin Variant ◽  
Hemoglobin F ◽  
Total Hemoglobin ◽  
Critical Residue ◽  
History Of ◽  
Unstable Hemoglobin

Abstract A new unstable hemoglobin, Hb Leslie, has been observed in three generations of a Georgia family. The propositus, a 42-yr-old black veteran with hemolytic anemia and splenomegaly, has a hemoglobin variant with an electrophoretic mobility similar to that of hemoglobin F. The variant comprises about 85% of the total hemoglobin and was isolated by chromatography. Chemical analysis has identified the abnormality as a deletion of the glutaminyl residue in position 131 (H9) of the beta-chain. Deletion of this critical residue which participates in the alpha1beta1 contact causes decreased stability of the hemoglobin without significant changes in functional properties or morphologic abnormalities in the erythrocyte. Family studies revealed hemoglobin Leslie occurring in combination with beta0-thalassemia, HbS, and HbC. All persons with the various Hb Leslie combinations, including the propositus, have no clinical manifestations other than anemia. In some the anemia is fully compensated. There is no history of drug- associated hemolysis.

Maternal Cigarette Smoking and Fetal Oxygen Transport: A Study of P50, 2,3-Diphosphoglycerate, Total Hemoglobin, Hematocrit, and Type F Hemoglobin in Fetal Blood

PEDIATRICS ◽  
1983 ◽  
Vol 72 (1) ◽  
pp. 22-26
Author(s):  
M. A. Bureau ◽  
D. Shapcott ◽  
Y. Berthiaume ◽  
J. Monette ◽  
D. Blouin ◽  
...  
Keyword(s):  
Cigarette Smoking ◽  
Carbon Monoxide Poisoning ◽  
Newborn Infants ◽  
Hemoglobin Concentration ◽  
Fetal Blood ◽  
Hemoglobin F ◽  
Maternal Cigarette Smoking ◽  
Total Hemoglobin ◽  
2,3 Dpg

The current study was undertaken to determine whether the O2 carrying capacity of newborns born to mothers who smoke can accommodate to carbon monoxide poisoning to compensate for the potential tissue hypoxia induced by CO. From a cohort of 1,222 women and newborn infants the total hemoglobin concentration, hematocrit, and carboxyhemoglobin (HbCO) were measured and analyzed with regard to the maternal HbCO level and the number of cigarettes smoked per day. Also, for 100 pairs of mothers, of whom 50 were smokers and 50 nonsmokers, and their newborns, measurements were made of the intraerythrocyte concentration of 2,3-diphosphoglycerate (2,3-DPG), the hemoglobin concentration, and the affinity of hemoglobin for oxygen by determination of O2 tension for 50% of Hb-O2 saturation (P50). The results showed no significant relationship between smoking habits of mothers and their levels of hemoglobin, P50, and 2,3-DPG; only the hematocrit was slightly increased in smoking mothers. However, in fetal blood a significant correlation was found between the number of cigarettes smoked per day and levels of hemoglobin, hematocrit, and P50. The P50 decreased in relationship to the increase in maternal HbCO. The P50 change was brought about by an increase in hemoglobin F found in the fetuses of mothers who smoked. Although all these changes were statistically significant, these mechanisms of acclimatization to CO poisoning appeared trivial in magnitude. It is concluded that the human fetus does not have a biologic capacity to accommodate to maternal cigarette smoking, and therefore the fetus is particularly susceptible to the adverse effects of cigarette smoking.

scholarly journals Leg ulcers in patients with sickle cell disease [see comments]

Blood ◽  
1989 ◽  
Vol 74 (4) ◽  
pp. 1403-1408 ◽  
Author(s):  
M Koshy ◽  
R Entsuah ◽  
A Koranda ◽  
AP Kraus ◽  
R Johnson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Ulcer Formation ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Hemoglobin C ◽  
Total Hemoglobin ◽  
Alpha Thalassemia

During the entry examination, leg ulcers were present in 2.5% of 2,075 patients 10 years of age and older with sickle cell disease who entered into the Cooperative Study of Sickle Cell Disease (CSSCD) between 1979 and 1986. Prevalence rates were highest among patients with sickle cell anemia and sickle cell anemia with thalassemia genotypes. Among sickle cell anemia patients free of ulcers at entry, the overall incidence was 5.73 per 100 person years in those having associated alpha-thalassemia and 9.97 for those without. Among sickle cell anemia patients with two alpha genes, the estimated incidence of leg ulcers is 2.38 per 100 person years and 6.12 per 100 person years among sickle cell anemia patients with three alpha genes (P less than .05). In both groups, the incidence was highest among those patients over 20 years of age and considerably higher among males than females (P less than .001). Leg ulcers were nonexistent in patients with sickle beta plus thalassemia and sickle hemoglobin C disease. Low steady-state hemoglobin is associated with a higher incidence of ulcer formation (P less than .0001) in sickle cell anemia patients. The protective effect of hemoglobin F is apparent at all levels of total hemoglobin among sickle cell anemia patients and those with associated alpha-thalassemia.

Effects of 5-aza-2′-deoxycytidine on fetal hemoglobin levels, red cell adhesion, and hematopoietic differentiation in patients with sickle cell disease

Blood ◽  
2003 ◽  
Vol 102 (12) ◽  
pp. 3865-3870 ◽  
Author(s):  
Yogen Saunthararajah ◽  
Cheryl A. Hillery ◽  
Don Lavelle ◽  
Robert Molokie ◽  
Louise Dorn ◽  
...  
Keyword(s):  
Cell Adhesion ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mechanism Of Action ◽  
Globin Gene ◽  
Fetal Hemoglobin ◽  
Red Cell ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Total Hemoglobin

Abstract Fetal hemoglobin (HbF) decreases polymerization of sickle hemoglobin (HbS) and improves outcomes in sickle cell disease (SSD). Therefore, a therapeutic goal in SSD is pharmacologic reactivation of HbF. Silencing of the γ-globin (HbF) gene is associated with DNA methylation. The cytosine analog 5-aza-2′-deoxycytidine (decitabine) hypomethylates DNA by inhibiting DNA methyltransferase. We examined if subcutaneous decitabine could increase HbF levels and improve SSD pathophysiology without cytotoxicity. Eight symptomatic SSD patients resistant or intolerant of standard treatment with hydroxyurea received decitabine 0.2 mg/kg subcutaneously 1 to 3 times per week in 2 cycles of 6-week duration. Treatment decreased neutrophils and increased mean HbF (6.5% to 20.4%, P < .0001) and mean total hemoglobin (76 to 96 g/L [7.6 to 9.6 g/dL], P < .001). Features of vaso-occlusive crisis pathophysiology such as red cell adhesion, endothelial damage, and coagulation pathway activity significantly improved. γ-Globin gene promoter methylation decreased, and platelets and the proportion of megakaryocytes and erythroid cells in the marrow increased without a decrease in marrow cellularity, consistent with a DNA hypomethylating, noncytotoxic mechanism of action. Weekly subcutaneous decitabine produces cumulative increases in HbF and total hemoglobin through a noncytotoxic mechanism of action. Chronic dosing and sustained increases in hemoglobin F and total hemoglobin levels may be possible. Further studies in SSD and thalassemia are indicated.

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