The Effect of Hydroxyurea on the Coagulation System in Sickle Cell Anemia and β-Thalassemia Intermedia Patients: A Preliminary Study

2003 ◽  
Vol 20 (6) ◽  
pp. 429-434 ◽  
Author(s):  
A. Koc ◽  
F. Gumruk ◽  
A. Gurgey
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Coagulation System ◽  
Thalassemia Intermedia ◽  
Preliminary Study

Evaluation Of Some Coagulation Profile (PT, APTT) In Sudanese Pregnant Women With Sickle Cell Anemia

2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Amged Hussein Abdelrhman ◽  
Abdelgadir Ahmed Abdelgadir
Keyword(s):  
Pregnant Women ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Case Control Study ◽  
Genetic Disorder ◽  
Clinical Manifestations ◽  
Coagulation System ◽  
Cell Disease ◽  
Duration Of Disease ◽  
Control Study

Background: Sickle cell disease refers to group of genetic disorder characterized by the predominance of hemoglobin S. Changes in the coagulation system seem to play an important role in the clinical manifestations of this disorder. Objective: This study aimed to determine the change in PT and APTT test in Sudanese pregnant women with sickle cell anemia. Material and methods: Fifty pregnant women with SCA with different age and different trimester, admitted to Mohammed Alamin Hamid hospital for children, were included case control study. Eleven healthy and pregnant women without SCA. Blood sample from three group were collected and investigated for PT and APTT. Results: The study revealed that in comparison with control mean PT (P=0.000) and APTT (p=0.000) high significant , in comparison with pregnant without SCA mean PT (P=0.000) and APTT (p=0.000) high significant ,no significant in comparison between all trimester mean PT (P=0.168) APTT (P=0.757) ,high significant in comparison with treatment mean PT(P=0,0000) APTT (P=0.000) ,in comparison with duration of disease and age mean PT(P=0.043) low significant with age APTT (P=0.558) no significant. Conclusion: The study concluded that these is hypercoagulable state in pregnant women with SCA indicated by prolongation in PT and APTT.

In Vitro and In Vivo Antioxidant Effect of Fermented Papaya Preparation (FPP) on Blood Cells of Beta-Thalassaemia Patients.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1766-1766
Author(s):  
Eitan Fibach ◽  
Johnny Amer ◽  
Ada Goldfarb ◽  
Eliezer Rachmilewitz
Keyword(s):  
Oxidative Stress ◽  
Lipid Peroxidation ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Peripheral Blood ◽  
Thalassemia Major ◽  
Oxidative Status ◽  
Beta Thalassemia ◽  
Thalassemia Intermedia

Abstract In sickle cell anemia (SCD) and thalassemia, although the basic lesions are mutations in the globin genes, the pathophysiology involves oxidative stress-mediated cell damage in the bone marrow (ineffective erythropoiesis due to apoptosis of early erythroid precursors) and in the peripheral blood (chronic hemolysis of mature RBC). In addition, some patients develop thromboembolic complications and recurrent bacterial infections, the etiology of which is related at least in part, to documented oxidative stress in platelets and neutrophils (PMN), respectively. To study the presence and the role of oxidative stress in thalassemia and SCD, we adapted flow cytometry techniques for measuring the generation of Reactive Oxygen Species (ROS), the content of reduced glutathione (GSH), membrane lipid peroxidation and externalization of phosphatidylserine (PS) moieties in RBC, platelets and PMN. Cells derived from the peripheral blood of patients with beta-thalassemia major, intermedia or SCD showed increased oxidative status (increased ROS, lipid peroxidation and PS externalization, and decreased GSH) compared with their normal counterparts. Incubating fresh blood samples from patients with thalassemia major and thalassemia intermedia with 10 mg/ml FPP for 16 hours at 37oC reduced the oxidative status of RBC as well as platelets and PMN. Experiments carried out in normal and thalassemic mice (Th3/+, a mouse model of human beta-thalassemia intermedia demonstrated that mice treated for one week with 10 mg/ml FPP (dissolved in the drinking water) had reduced oxidative stress compared to control mice. The in-vivo effect of FPP was tested on 9 patients with beta-thalassemia (6 - major and 3 - intermedia) treated with 3 gr FPP per os three times a day for 12–15 weeks. Following the treatment, the ROS in RBC, platelets and PMN decreased and the GSH increased in all patients (see table). Six of these patients responded by a modest increase in RBC, reticulocytes and hemoglobin levels. These results suggest that FPP may have an important clinical efficacy as an antioxidant in thalassemia and sickle cell anemia. The in vivo effect of FPP treatment of beta-thalassemia patients Baseline After treatment n Mean ± SE Mean ± SE P-value* * Paired samples t-test RBC 9 324.07 ± 29.19 209.55 ± 23.65 0.001 ROS Platelets 9 223.73 ± 26.49 109.11 ± 8.71 0.001 PMN 9 222.72 ± 46.42 117.61 ± 8.98 0.045 RBC 9 55.37 ± 5.37 94.88 ± 3.71 0.001 GSH Platelets 9 59.41 ± 4.98 97.55 ± 5.26 <0.0001 PMN 9 58.29 ± 5.35 90.06 ± 5.87 0.005

Preliminary Study of Coping, Perceived Control, and Depressive Symptoms in Youth with Sickle Cell Anemia

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Kemar V. Prussien ◽  
Rachel E. Siciliano ◽  
Abagail E. Ciriegio ◽  
Chelsea A. Lee ◽  
Michael R. DeBaun ◽  
...  
Keyword(s):  
Depressive Symptoms ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Perceived Control ◽  
Preliminary Study

Plasma ‘Free’ HB Is Related to Red Cell Derived Vesicle Numbers in Sickle Cell Anemia and Thalassemia Intermedia: Implications for Nitric Oxide (NO) Scavenging and Pulmonary Hypertension.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1671-1671 ◽  
Author(s):  
Maxwell Westerman ◽  
Arnold Pizzey ◽  
Jocelyn Hirschman ◽  
Mario Cerino ◽  
Ada Eze ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Healthy Adult ◽  
Thalassemia Intermedia ◽  
Plasma Hemoglobin ◽  
Increased Risk ◽  
The Relationship ◽  
Normal Controls

Abstract Plasma hemoglobin (Hb) is a scavenger of nitric oxide (NO), which is a likely contributor to the pathogenesis and treatment of clinical abnormalities such as pulmonary hypertension and vasocclusive episodes in sickle cell anemia (SCA) and possibly in thalassemia syndromes, where pulmonary hypertension has been described most frequently in splenectomised patients with thalassemia intermedia (TI). Since plasma Hb consists of both free Hb and RBC-derived microvesicle Hb (Greenwalt. Vox Sang1991;61:14), the relationship between plasma Hb and circulating vesicles may be significant. We have measured plasma Hb and plasma vesicle levels in adults with SCA and thalassemia intermedia (TI). Patients with SCA were all untransfused and TI patients had no transfusions during the previous 3 months. Plasma Hb values in healthy adult controls (1.77±0.2, n=7) were significantly lower than in SCA (11.21±2.08mg/dl, n=15, p=0.003) or in splenectomised TI patients (48.46 ±3.66mg/dl, n=5, p=0.0038). Plasma Hb levels were significantly greater in TI as compared to levels in SCA (p=0.001). Vesicle numbers in SCA were 12.59±3.65 x103/ul (n=21, p< 0.001, SCA v control) and in TI were 24.19 ±12.23 (n=7, p< 0.001, TI v control). Thus both plasma Hb and circulating vesicle levels in SCA and TI were significantly greater than healthy controls. Plasma Hb was significantly greater in TI than in SCA and circulating vesicles markedly greater in TI than in SCA. Furthermore there was a significant correlation between plasma Hb and vesicle numbers in SCA, TI and normal controls (n=26, R2=0.59, p=0.0015). An analysis of splenectomised versus non-splenectomised TI patients revealed a further trend; both plasma Hb and vesicle numbers were significantly higher in splenectomised (n=5, n=7 respectively) than in non-splenectomised patients (n=4, n=4 respectively). Plasma Hb and vesicle numbers were respectively 48.5±3.6 and 24.19±12.2 in splenectomised patients compared to 17.18±5.58 (p=0.014) and 4.36±0.81 (p=0.008) in non-splenectomized TI patients. These findings show that raised plasma Hb levels are related to increments in vesicle numbers in TI and SCA. Splenectomy in TI, which is associated with increased risk of pulmonary hypertension and thrombosis, is associated with increased vesicle numbers and plasma Hb. We suggest that the scavenger characteristics of Hb containing vesicles for NO may differ from the scavenger characteristics of free Hb and thus require detailed study..

Plasma Hemoglobin: Potential Sources.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3814-3814
Author(s):  
Maxwell Westerman ◽  
Arnold Pizzey ◽  
Jocelyn Hirschman ◽  
Mario Cerino ◽  
Yonit Well-Weiner ◽  
...  
Keyword(s):  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Lactic Dehydrogenase ◽  
Thalassemia Intermedia ◽  
Intravascular Hemolysis ◽  
Plasma Hemoglobin ◽  
Potential Sources

Abstract Plasma hemoglobin(Hb) is a measure of circulating red blood cell(RBC) destruction and is considered to be the basic indicator of intravascular hemolysis. We have examined the effects of splenectomy on levels of plasma Hb and circulating RBC-derived vesicles in patients with thalassemia intermedia (TI) and compared the results to patients with sickle cell anemia (SCA). Plasma Hb levels in splenectomized patients with TI were 48.5 ± 3.7 mg/ml (5)(Mn ± SEM)(No.of patients) and vesicle levels were 11.29 ± 1.12 x 10 3 /ul blood (9). In contrast, plasma Hb levels in patients with SCA were (14.52 ± 3.29)(21) and vesicle levels were 13.2 ± 2.57)(34). Plasma Hb levels and vesicle levels are closely associated in TI and SCA (r=0.79, p=0.01[9]; r=0.58, p=0.006[21] respectively). The finding that plasma Hb levels in patients with TI and SCA, both asplenic, differ in their relationships to corresponding and similar vesicle levels, suggests that other hemolytic factors may contribute to plasma Hb levels. Of importance would be intramedullary hemolysis which is considerable in TI. Vesiculation, which may occur with intramedullary hemolysis does not appear to contribute to circulating vesicle levels. The ratio of plasma Hb levels to vesicle counts would be a marker to distinguish intramedullary hemolysis from intravascular hemolysis. Similar considerations may apply to measures of lactic dehydrogenase (LDH) which is also an indicator of RBC destruction and intravascular hemolysis. The findings suggest that the contribution of intramedullary hemolysis as well as the contribution of intravascular hemolysis should be considered in measurements of plasma Hb.

The orientation of sickle hemoglobin fibers within fascicles

1988 ◽  
Vol 46 ◽  
pp. 400-401
Author(s):  
Christopher A. Miller ◽  
Bridget Carragher ◽  
William A. McDade ◽  
Robert Josephs
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Relative Orientation ◽  
Unit Cell ◽  
Red Cell ◽  
High Ph ◽  
Sickle Hemoglobin ◽  
Polar Crystal ◽  
Helical Configuration

Highly ordered bundles of deoxyhemoglobin S (HbS) fibers, termed fascicles, are intermediates in the high pH crystallization pathway of HbS. These fibers consist of 7 Wishner-Love double strands in a helical configuration. Since each double strand has a polarity, the odd number of double strands in the fiber imparts a net polarity to the structure. HbS crystals have a unit cell containing two double strands, one of each polarity, resulting in a net polarity of zero. Therefore a rearrangement of the double strands must occur to form a non-polar crystal from the polar fibers. To determine the role of fascicles as an intermediate in the crystallization pathway it is important to understand the relative orientation of fibers within fascicles. Furthermore, an understanding of fascicle structure may have implications for the design of potential sickling inhibitors, since it is bundles of fibers which cause the red cell distortion responsible for the vaso-occlusive complications characteristic of sickle cell anemia.

Abstract #914: Polyglandular Autoimmune Syndrome in a Patient with Sickle Cell Anemia and Iron Overload

2005 ◽  
Vol 11 ◽  
pp. 85
Author(s):  
Allison Elise Kerr ◽  
Wolali Odonkor ◽  
Gail Nunlee-Bland ◽  
Juanita Archer ◽  
Anitha Kolukula ◽  
...  
Keyword(s):  
Iron Overload ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Syndrome
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