Microparticle Profile During Painful Crisis and Steady State Period In Sickle Cell Anemia

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4681-4681
Author(s):  
Anil Atmis ◽  
Ilgen Sasmaz ◽  
Bulent Ali Antmen ◽  
Keyword(s):  
Tissue Factor ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Complete Blood Count ◽  
Conflicts Of Interest ◽  
Systemic Disease ◽  
Control Group ◽  
Healthy Children ◽  
Crisis Period ◽  
Painful Crisis

Introduction Sickle cell anemia is a disease which is characterized with hemolytic anemia, hypercoagulopathy and painful crisis. Microparticles are 0,1-1 µm sized little membrane particles which are derived during activation or apoptotic phase of cell cycle. It is reported that microparticles are increased in many systemic disease including sickle cell anemia. Aim In this study we aimed to investigate the role of microparticles on during crisis and non-crisis periods in sickle cell anemia patients. Materials and method Twenty nine patients, following by Cukurova University, Department of Pediatric Hematology, are included in this study. Blood samples were collected in 26 of these patients in non-crisis period. Control group formed with 18 healthy children without any systemic disease. Complete blood count, hemoglobin electrophoresis and biochemical parameters were studied in both groups. Also patients’ total microparticle levels, erythrocyte (CD235a), endothelial (CD106), monocyte (CD14) particle levels and tissue factor expressing (CD142) microparticle levels were studied by flow cytometry and whole data was statistically analyzed. Findings Hemoglobin and hematocrit levels were significantly low in sickle cell anemia patients (p<0,001). Levels of HbS were significantly high during crisis period comparing with mean HbS levels during non-crisis period (p<0,001). Total microparticle levels were significantly high in sickle cell anemia patients with painful crisis comparing with control group (p<0,05). Erythrocyte and monocyte microparticle levels were significantly high in patients with painful crisis comparing with non-crisis periods (p<0,05). Endothelial and tissue factor expressing microparticle levels were high in patiens with crisis comparing to non-crisis period but this was not statistically significant (p>0,05). There was not any significant relation with frequency of crisis and microparticle levels (p>0,05). Microparticle levels were low in patients whose were taking hydroxiurea treatment comparing with non-hydroxiurea treatment but this data was not statistically significant (p>0,05). Result As a result we found high levels of total microparticle, erythrocyte and monocyte microparticles in sickle cell anemia patients during painful crisis period. This important clue of crises was need further studies in order to understand the effect of microparticles on pathophysiology of sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Elevated End-Tidal Carbon Monoxide Concentration in Children with Sickle Cell Anemia

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1390-1390
Author(s):  
Ashutosh Lal ◽  
Kristen Yen ◽  
Lasandra Patterson ◽  
Alisa Goldrich ◽  
Anne M Marsh ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Young Children ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Second Hand Smoke ◽  
Control Group ◽  
Exhaled Breath ◽  
Healthy Children ◽  
The Mean ◽  
End Tidal

Abstract Background: Carbon monoxide (CO) produced during oxygen-dependent cleavage of porphyrin ring of heme is excreted in exhaled breath. The catabolism of heme is increased when red blood cells are destroyed at an accelerated rate. Thus, quantifying CO in exhaled breath could serve as an indicator of hemolysis. However, the requirement for forced breath sample has limited the measurement of exhaled CO in young children. Objective: To assess end-tidal CO concentration (ETCOc) in children with sickle cell anemia (SCA). Design/Methods: ETCOc was measured using the CoSense ETCO Monitor (Capnia Inc. Palo Alto, CA). Children between 5-14 years with SCA (Hb SS) who were not on chronic transfusions were eligible. Healthy children served as age-matched controls. Children with exposure to second-hand smoke, acute respiratory infection or symptomatic asthma were excluded. End-tidal breath samples were collected by placing the tip of a nasal cannula 5 mm into the nares. Up to 3 measurements were taken for each subject and the highest ETCOc value was used for analysis. (ClinicalTrials.gov: NCT01848691) Results: The mean (range) age of 16 children with SCA and 16 controls was 9.7 years (5-14 years) and 9.9 years (5-14 years), respectively. The mean (± s.d.) ETCOc for SCA was 4.85 ± 2.24 ppm versus 0.96 ± 0.54 ppm for control group (p<0.001). The ETCOc in the control group ranged from 0.2 to 2.3 ppm, but was ≤1.2 ppm in 14/16, which is suggested as the upper limit of normal for healthy children. In the SCA group, the ETCOc range was 1.8 to 9.7 ppm, with values ≥2.4 ppm in 15/16 subjects. A threshold ETCOc value of >2.1 ppm provided both sensitivity and specificity equal to 93.8% (69.8-99.8%) for distinguishing SCA from healthy children. Children with SCA who had higher absolute reticulocyte count also demonstrated higher ETCOc (r=0.62, p=0.011). Patients with severe anemia (hemoglobin <8 g/dL) had a higher mean ETCOc (5.43 ppm) than the rest (4.40 ppm) but the difference was not significant. ETCOc level tended to increase with age in SCA (r=0.45, p=0.08). Conclusions: Carbon monoxide in exhaled breath can be measured in young children in the clinic using a portable monitor. ETCOc may be a valuable tool for non-invasive monitoring of the severity of hemolysis in SCA. The mean ETCOc was 5-fold higher in SCA compared with controls, with little overlap seen between the groups. This suggests a potential use for ETCOc as a point-of-care screening test for SCA in children. Figure 1 Figure 1. Figure 2 Figure 2. Disclosures Lal: Capnia, Inc: Research Funding. Yen:Capnia, Inc. : Employment. Bhatnagar:Capnia, Inc: Employment.

The Eveluation of Pulmonary Functions in Children with Sickle Cell Anemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4628-4628
Author(s):  
Cem Kurt ◽  
Ilgen Sasmaz ◽  
Bulent Antmen ◽  
Yurdanur Kilinc ◽  
Sadi Kurdak ◽  
...  
Keyword(s):  
Patient Group ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Exercise Test ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Healthy Children ◽  
Diffusion Test ◽  
Pulmonary Functions

Abstract Abstract 4628 Aim In this study we evaluated to pulmonary functions and determined relations of these findings with clinical parameters in children with sickle cell anemia (SCA) who were at follow up in our pediatric heamatology clinic. Materials and Methods 24 children with sickle cell anemia and 9 healthy children as control group include to the study. Complete blood count, hemoglobin electrophoresis and biochemical values were eveluated for both groups. At pulmonology department, the carbonmonoxide diffusion test performing for both groups. At the same day spirometric respiratory function evaluation and exercise test performed both groups at department of sports physiology. The data recieved are compared statistics. Results HbS, HbF, SGPT, ferritin, total bilirubine, direkt bilirubine and Fe++ values were high at patient group (p<0.05). Hemoglobin and hematocrit values were low at patient group according to control group as expected (p<0.05). The number of SCA patient who had one-three venoocclusive crises (VOC) were 14 (58.3%), patient who had three or more VOC were 7 (29.2%) and patient who had no VOC were 3 (12.5%). The number of patient who had acute chest syndrome (ACS) were 5 (20.9%) and 19 patients had no ACS (79.1%). Ýmpaired isole carbonmonoxide diffusion test was established at the 62.5% of the patient's. At patient group, spirometric FEV1 and MEF25 measurement were found lower than the control group (p<0.05). At exercise test VO2/HR rate were lower for patient group (p<0.05). VE/VO2 rate (p=0.023) and R (p=0.016) measurement were found higher. Conclusion Pulmonary gas transfer was found difficult in patients with SCA. Respiratory airways established obstructed in spirometric evaluation. Obstructive defficiensies have to be follow up. Oxygen pulse and respiratory exchange rates were determined low and more oxygen usage was observed for aerobic metabolic activity. With these results, ýt can be say that chronic inflamation process at lung due to oxygen radicals and hipoksemia in sickle cell patients, the aerobic respiratory load was increased. Disclosures: No relevant conflicts of interest to declare.

Plasma Protein Oxidation and Triglyceride Levels in Sickle Cell Anemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2578-2578
Author(s):  
Yesim Oztas ◽  
Selma Unal ◽  
Nuriman Ozgunes
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell ◽  
Plasma Protein ◽  
Sickle Cell Anemia ◽  
Protein Oxidation ◽  
Conflicts Of Interest ◽  
Protein Carbonyl ◽  
Healthy Children ◽  
Healthy Controls ◽  
Lipid Balance

Abstract Abstract 2578 Poster Board II-555 Introduction: A mutant and unstable hemoglobin is characteristic in sickle cell anemia (SCA) with an increased formation of methemoglobin, hemichrome and haemin. Increased oxidative stress and altered lipid balance in the plasmas of SCA patients have been documented previously. Lower total plasma cholesterol (TC) and higher-than-avarage triglyceride (TG) levels have also been reported in SCA patients compared to healthy controls. Plasma phospholipids (PL) were normal or increased according to various studies. However, to our knowledge only a few reports documented the plasma protein oxidation in SCA patients. In this study protein oxidation, lipid levels, bilurubin levels and their probable correlation were investigated in the plasmas of SCA patients, carriers and healthy controls. Patient and Methods: 15 SCA patients, 10 carriers and 10 healthy controls were included in the study. The patients had not been transfused and hospitalized and had no crisis for the last three months. The oxidation status of plasma and its proteins were detirmined by total sulfhydryl (T-SH) and protein carbonly levels respectively. TC, TG and bilurubin levels were detirmined in the plasma and PL levels were detirmined in the lipid extract from plasma. Results: SCA patients had lower T-SH levels compared to controls (p=0.003) and higher protein carbonyl levels compared to carriers (p=0.001) and healthy children (p=0.032). T-SH values were 417.41±77.90 μM, 457.46±106.26 μM, 522.59±77.18 μM and protein carbonyl levels were 0.93±0.15 nmol/mg protein, 0.74±0.08 nmol/mg protein , 0.82±0.05 nmol/mg protein for patients, carriers and controls respectively. Significantly lower mean TC levels than controls (p<0.0001) and higher TG levels than carriers (p=0.001) were observed in SCA patients. TC levels were 116.67±23.60, 127.53±22.40, 151.62±16.53 and TG levels were 89.31±23.50 mg/dl, 60.94±9.18 mg/dl, 82.96±21.06 mg/dl for patients, carriers and controls respectively. Phospholipid levels were not different between the groups. TG levels were positively correlated to protein carbonyl levels (p=0.002) and to bilirubin levels (p=0.033). Bilurubin levels and protein carbonyl levels are also positively correlated (p<0,0001). Conclusions: Alteration of lipid balance besides protein oxidation and hemolysis were observed in the plasmas of SCA patients compared to the carriers and healthy group. The positive correlation between total bilurubin, TG and carbonyl levels indicates that oxidative stress may affect the SCA plasma extensively, ie. lipids, proteins and erythrocytes are all affected. Disclosures: No relevant conflicts of interest to declare.

scholarly journals The Effect of Ketamine Administration on Pain Control in Painful Crisis of Sickle Cell Anemia Patients during Childhood: A Retrospective Observational Study

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4919-4919
Author(s):  
Yurdanur Kilinc ◽  
Hacer Temizturk ◽  
Hayri levent Yılmaz ◽  
Metin Cil ◽  
Goksel Leblebisatan ◽  
...  
Keyword(s):  
Side Effects ◽  
Pain Score ◽  
Sickle Cell ◽  
Drug Administration ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
Vital Signs ◽  
Number Of Patients ◽  
The Mean ◽  
Painful Crisis

Abstract Background: Sickle cell anemia (SCA) is a hemoglobinopathy as a result of substitution of glutamic acid with valine in the 6th position of the β globin chain. The vaso-occlusive crises of patients are often caused by pain that requires in-patient treatment. The attack rate is reported to changed between 0.5-1.0 attacks per year per patient. It could be reach up to 10% in 5% of patients. Identification of the SCA painful crisis and the determination of appropriate treatment is very important for the pain relief. Recently studies support that ketamine treatment is beneficial in patients who do not respond to traditional methods. Ketamine is a non-competitive antagonist at the NMDA receptor. We used retrospective visual analogue scale (VAS) scoring system in acute painful crisis patients and then applied ketamine and tramadol to investigate the effect of treatment and side effects and the maximum change in pain score within the first hour after drug administration retrospectively. The aim of this study is evaluate the level of ketamine effect in painful crises. Methods: Patients older than 6 years of age with acute painful crisis, defined as VAS of greater or equal to 4 were enrolled.The retrospective data was collected from electronic records and included age, sex, vital signs, changes in pain score within 1 hour, and side effects. Ketamine (0.25 mg / kg / dose) and tramadol (0.1-0.4 mg / kg / h) were administered. Patients with a pain score of <4 or a decrease of ≥3 points in VAS were considered to be responsive. Results: Ketamine group (n = 16), 6 female (37.5%), 10 male (63.5%) and the mean age was 15.4 (7-21) years. Tramadol group (n = 31) 15 female (48%), 16 male (52%) and the mean age was 15.6 (6-21) years. The age and sex groups were similar (p> 0.05). After drug administration, vital signs were observed in all patients in normal range to their age. In the tramadol and ketamine groups, the number of patients who responded at 20 minutes were 9(29%)-9(56%), 12(38%)-11(68%) (p<0.05) at 60 minutes. Sixteen patients (51.6%) responded in the 6th hour of the tramadol group, no evaluate in ketamin group. No life-threatening side effects was observed in any patient. Conclusion: In the treatment of painful crises, ketamine administration was found to be more effective than tramadol in the first hour. Due to the longer duration of the initial effect of tramadol treatment, ketamine should be considered as an option in the rapid treatment of painful crises. Disclosures No relevant conflicts of interest to declare.

The Value of Procalcitonin and Lipopolysaccharide Binding Protein to Differentiate the Fever In the Patients with Sickle Cell Anemia

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4818-4818
Author(s):  
Selma Unal ◽  
Ali Ertug Arslankoylu ◽  
Necdet Kuyucu ◽  
Gönül Aslan ◽  
Semra Erdogan
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Binding Protein ◽  
Control Group ◽  
Healthy Children ◽  
Lipopolysaccharide Binding Protein ◽  
Wbc Count ◽  
Serum Crp ◽  
And Control ◽  
Lipopolysaccharide Binding

Abstract Abstract 4818 Objective: Differentional diagnosis of fever is very important in patients with sickle cell anemia (SCA) in order to prevent inappropriate antibiotic use, drug resistance and to shorten the hospitalization period. A reliable marker to be used in the differentional diagnosis of fever in these patients has not been defined yet. We aimed to evaluate the values of C Rective Protein (CRP), procalcitonin (PCT), and lipopolysaccharide binding protein (LBP) levels in the differentional diagnosis of fever in patients with SCA. Material and Methods: 86 children with SCA (40 males and 36 females, mean age of 9.6 ± 3.84, range: 1–18 years), (Group 1) and 49 healthy children as a control group (mean age: 8.8 ± 3.91, range: 1–18 years) (Group 2) were included in this study. Patients who had admitted to the emergency department for concurrently vasoocclusive crisis and fever (axillary temperature ≥38C°) were classified as Group 1A and who had vasoocclusive crisis but no fever were classified as Group 1B and the patients without fever and vasoocclusive crisis were classified as Group 1C. A detailed history was taken from every child, and a full physical examination was performed. The patients who had taken antibiotics in last one week were excluded. The type of vasoocclusive crisis were recorded in Group 1A and 1B patients. In Group 1A patients, the fever was evaluated with appropriate laboratory tests (WBC count, periferic blood smear, serum CRP level, urinary test, chest radiography, blood and urinary culture). No infection focus was identified in patient and conrol groups. The WBC count, serum CRP, PCT and LBP levels were evaluated in all the patient and control groups. Results: The median CRP level of all patient groups was significantly higher than the control group (0.78 mg/L; range 0.21–70.0) (p<0.0001). The median CRP level in Group 1A (7.42 mg/L) and Group 1B (6.94 mg/L) were significantly higher than group 1C patients (2.24 mg/L). There were no significant difference between the SCA groups considering median serum PCT levels (Group 1A: 0.18 ng/ml, Group 1B: 0.11 ng/ml, Group 1C: 0.13 ng/ml, p>0.05). These values were significantly higher than control group (0.08 ng/ml) (p<0.0001). LBP level in Group 1A (median:11.5 μg/ml) was significantly higher than Group 1B (median: 8.9μg/ml), Group 1C (median: 7.7μg/ml) and control group (median:5.9μg/ml) (p<0.0001). Also serum LBP in both Group 1B and Group 1C were higher than control group (p<0.0001). Conclusion: PCT level is not affected from the acute inflamation originates from the vasoocclusive crisis. Thus, serum PCT level can be considered a good marker in the differentional diagnosis of fever in patients with SCA. Disclosures: No relevant conflicts of interest to declare.

scholarly journals CLINICAL SIGNIFICANCE OF ASSESSMENT OF THROMBOSPONDIN AND PLACENTA GROWTH FACTOR LEVELS IN PATIENTS WITH SICKLE CELL ANEMIA: TWO CENTERS EGYPTIAN STUDIES

2014 ◽  
Vol 6 (1) ◽  
pp. e2014044 ◽  
Author(s):  
Adel Abd elhaleim Hagag ◽  
Ghada Elmashad ◽  
Aml Ezzat Abd El-Lateef
Keyword(s):  
Growth Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
University Hospital ◽  
Control Group ◽  
Healthy Children ◽  
Cell Disease ◽  
Placenta Growth Factor ◽  
Worldwide Distribution

Abstract: Background: Sickle cell disease has a worldwide distribution. Vaso-occlusive crisis (VOC) is one of the most important clinical features of the disease. Thrombospondin (TSP1) and Placenta growth factor (PlGF) have been reported to be involved in sickle cell diseases (SCD). Objective: The aim of this study is to assess TSP1 and PlGF levels in SCD patients. Patients and methods: This study was carried out in forty patients with sickle cell anemia who were attendants to Hematology units, Pediatric Departments, Tanta and Elmenofia University Hospital in the period between December 2011 and December 2013 including twenty patients with sickle cell anemia during vaso-occlusive crisis (twelve males and eight females) and twenty patients with sickle cell disease out of crisis (ten males and ten females). Also this study included twenty healthy children of matched age and sex as a control group. Serum TSP1 and PlGF levels were analyzed by ELISA. Results: Mean serum TSP1 levels were significantly higher in SCA patients with crisis than SCA patients out of crisis and were significantly higher in SCA patients with or without crisis compared to controls. Mean serum Placenta growth factor levels were significantly higher in SCA patients with crisis than SCA patients out of crisis and were significantly higher in SCA patients with or without crisis compared to controls. Conclusions: TSP1and PlGF concentration may be useful VOC markers in SCD patients. Recommendation: Further studies should be conducted to determine the exact point before VOC, when serum TSP1 and PIGF levels begin to increase. This requires monitoring of the TSP1 and PIGF levels in sickle cell patients out of crisis, showing how rapidly these levels increase just before VOC development.

scholarly journals Longitudinal Analysis of Hydroxyurea and Fetal Hemoglobin in Adult Patients with Sickle Cell Anemia

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2054-2054
Author(s):  
Aryeh Pelcovits ◽  
Giancarlo Riotto ◽  
Katie Cherenzia ◽  
Patrick T. McGann ◽  
John L Reagan
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
Surrogate Marker ◽  
Fetal Hemoglobin ◽  
P Value ◽  
Significant Difference ◽  
Dosing Strategies ◽  
Over Time ◽  
Painful Crisis

Abstract Introduction: Hydroxyurea (HU) was the first FDA-approved therapy for sickle cell anemia (SCA) and remains the most well-established disease-modifying therapy, reducing painful crisis and improving morbidity and mortality. HU improves outcomes through the upregulation of fetal hemoglobin (HbF). Dosing is highly variable with doses ranging from &lt;10-35 mg/kg/day. Most dosing strategies escalate with a goal of mild myelosuppression, commonly defined using an absolute neutrophil count (ANC) between 2,000-4,000 and platelets &gt;80,000. Macrocytosis is often used as a surrogate marker for compliance and effect. Despite its well-established effectiveness, clinical use remains limited with only 12% of adults with SCA taking HU. The reasons for this are multifactorial but include skepticism by both providers and patients regarding its effectiveness in the adult population. Some experts suggest up to 30% of adults are non-responders with no significant HbF change, and many believe that the effect of HU fades with time. Dosing strategies are usually quite conservative to prevent excessive myelosuppression, though dose is highly correlated with clinical effect and ultimate %HbF. There is very limited real-world data regarding long term effectiveness of HU in adults with SCA over time. Methods: We retrospectively analyzed the records of the 109 adults with SCA currently treated in our multidisciplinary sickle cell clinic. Data from 1/1/2011-6/30/2021 was collected, including genotype, HU prescription status, current and maximum laboratory values (HbF, MCV, ANC), and number of admissions for painful crisis. We performed Wilcoxon rank sum testing between pts prescribed and not prescribed HU and measures of HbF (peak, average and current) and number of admissions for painful crisis over the study period. Results: Among 106 pts (3 excluded from analysis, 2 for lack of data and 1 for post-transplant status), 79 are currently prescribed HU (75%). Among our 63 pts with HbSS genotype 58 are prescribed HU (92%). Average HbF over time for all pts prescribed HU was 11.9%. Average peak HbF was 18.1% and average current HbF is 12.4%. In the pts not prescribed HU HbF average, peak and current levels were 5.5%, 6.7%, and 5.0% respectively. HU prescription was significantly associated with increased HbF at peak, average, and current levels (p-value &lt;0.001, Figure A). HU prescription was also significantly associated with decreased number of admissions for painful crisis (p-value 0.001). Among pts prescribed HU, MCV levels &gt;100 at time of peak HbF were associated with higher peak levels than pts with MCV &lt;100 (p-value &lt;0.001, Figure B). Larger peak HU doses were also correlated with higher MCVs at the time of peak HbF levels (Figure C). Larger current HU doses were also significantly associated with higher current HbF levels (Figure, D). Doses &gt;9.9m/kg were associated with significantly higher HbF levels however there was no significant difference between dose levels 10-19.9mg/kg and 20-29.9 or 20-29.9 and 30-39.9mg/kg (p-values 0.02, 0.68 and 0.84 respectively). Among pts prescribed HU, 34 obtained ANC levels &lt;4000 at the times of peak HbF and 24 at the current dosing level (43% and 30% respectively). 24 pts obtained both MCV&gt;100 and ANC &lt;4000 at time of peak HbF and only 11 achieved both at current dosing levels (30% and 14% respectively). Conclusion: In our adult multidisciplinary sickle cell clinic prescribing rates are well above current HU usage for adults with SCA. Our data notes that elevated HbF levels can be maintained over long periods of time. HU prescription was significantly associated with increased HbF levels and reduced admissions for painful crisis. This was despite a majority of pts not meeting target prescribing levels of ANC &lt;4000 and MCV &gt;100. While higher peak HbF levels were significantly associated with higher HU doses, this was only true for doses above 9.9 mg/kg. Further investigation is needed to explore the factors contributing to suboptimal HbF and MCV response, including careful assessment with medication adherence and dose selection. Overall, these data illustrate the importance of dosing and suggest that one size dosing of HU for adults with SCA does not fit all. We hypothesize that there may be a role individualized dosing strategies in adults with SCA, incorporating factors such as pharmacokinetics and renal function to achieve the optimal dose for each patient. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Study of transcranial Doppler ultrasound and endothelin-1 in children with sickle cell anemia: a single-center study

2021 ◽  
Vol 20 (3) ◽  
pp. 31-35
Author(s):  
S. Ragab ◽  
E. Badr ◽  
H. El-Kholy ◽  
M. El-Hawy
Keyword(s):  
High Risk ◽  
Patient Group ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Control Group ◽  
Healthy Children ◽  
Endothelin 1 ◽  
The Impact

To assess the impact of our transcranial Doppler (TCD) screening program on the incidence of a first stroke in children with sickle cell anemia and to study the role of elevated serum endothelin-1 (an inflammatory mediator) in these children. Background: stroke is a major complication of sickle cell disease (SCD), even in very young children. About 11% of children with homozygous sickle cell anemia (SS) develop stroke by the end of the second decade of life. The underlying etiology in most cases is an ischemic stroke caused by large-vessel stenosis or occlusion. Transcranial Doppler (TCD) recommended as a routine screening test to identify children at high risk of developing a stroke, measures flow velocities within large intracranial arteries. TCD should be routinely performed in children between 2 and 16 years as this age group is at the highest risk of sickle cerebral vasculopathy. We carried out a prospective case-control study which included 2 groups: a patient group consisted of 30 children with sickle cell anemia and sickle thalassemia and a group of 30 healthy children of matched age and sex. Each group included 11 males (36.5%) and 19 females (63.5%); the age range was 2 to 17 years. Both groups underwent a thorough clinical examination and laboratory tests (CBC, liver and renal function, serum ferritin and endothelin-1). Additionally, TCD was performed in all children included in the patient group. According to the results of TCD, time-averaged mean of the maximum velocity (TAMMX) was < 170 cm/s (normal), 170–200 cm/s (conditional), ≥ 200 cm/s (high risk) in 20 (66.7%), 4 (13.3%) 6 (20%) patients, respectively. The level of endothelin-1 was significantly higher in the patients (57.1 ± 91.3) than in the controls (21.9 ± 14.8). Hemoglobin concentration was significantly lower in the patient group than in the control group, but the levels of reticulocytes, WBCs and serum ferritin were significantly higher in the patients than in the healthy controls. Serum Endotheline-1 level was higher in patients with sickle cell anemia than control group.

Steady State Sickle Cell Anemia Is Associated with Increased Formation of Erythrocyte-Derived Microparticles and Acceleration of Thrombin Generation.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4001-4001 ◽  
Author(s):  
Mourad Chaari ◽  
Katia Stankovic ◽  
Vasso Galea ◽  
Francoise Robert ◽  
Amir Khaterchi ◽  
...  
Keyword(s):  
Steady State ◽  
Plasma Concentration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Plasma Levels ◽  
Thrombin Generation ◽  
Conflicts Of Interest ◽  
Inverse Correlation ◽  
Control Group ◽  
Generation Process

Abstract Abstract 4001 Poster Board III-937 Introduction Patients with Sickle Cell Anemia (SCA) are at risk of thrombosis, but this clinical manifestation is variable and it is probably not associated with the frequency of vaso-occlusive crisis. Increased plasma levels of platelet- and erythrocyte-derived microparticles and hypercoagulability markers have been reported in steady state SCA patients. However, the link between hypercoagulability and SCA is not completelty understood. Aim of the study We determined erythrocyte-derived (Ed-MP) and platelet-derived microparticles (Pd-MP) levels in patients with steady state SCA. We studied their relationship with hemolysis markers and their impact on thrombin generation process. Materials and methods Consecutive out-patients with steady state SCA (n=78) and 20 healthy age and sex-matched controls were included. They were free of any acute episode of SCA for at least one month prior inclusion. Microparticles were assessed with standardized whole blood flow cytometry assay. Ed-MP and Pd-MP were identified using respectively anti-CD235a and anti-CD41 monoclonal antibody and annexine V. Thrombin generation (TG) in citrated platelet poor plasma was assessed with Calibrated Automated Thrombogram® (Stago, France) using PPP-reagent 5pM® (Thrombinoscope BV, Nederlands). The following TG parameters were analyzed: lag-time (LT), time to peak of thrombin (ttpeak), peak of thrombin (peak), mean velocity rate index of the TG (MRI) and endogenous thrombin potential (ETP). Results Mean patient age was 25±8 (range 17-58 ys). In the patients group, Ed-MP and Pd-MP, expressing or not phosphatidyl-serine (PS), were significantly increased compared to the control group. Thrombogram parameters were not significantly different in both groups (Table 1). There was a slight though significant inverse correlation between Ed-MP and both LT and ttpeak (r=-0.235, r=-0.315 respectively; p<0.05). Ed-MP levels were correlated with MRI increase (r=0.241; p<0.05). Ed-MP values were inversely correlated with Hb levels and well correlated with reticulocytes count (r=-0.427, r=0.520 respectively; p<0.05). No relationship was found between Ed-MP and ETP values. The sub-population of Ed-MP/PS+ (expressing PS) showed also an inverse correlation with both ttPeak (r=-0.315, p<0.05) and ETP (r=-0.236; p<0.05), and a positive correlation with MRI (r=0.306 ; p<0.05). Pd-MP concentration was inversely correlated with Hb levels (r=-0.273 ; p<0.05). Only Pd-MP/PS+ plasma concentration was slightly by significantly correlated with ETP (r=0.225; p=0,049). Conclusion Patients with steady state SCA presented a significant increase of Ed-MP and Pd-MP plasma levels which seems to be linked to haemolysis degree. Each type of microparticles had different impact on TG process. Ed-MP induced acceleration of TG kinetics without increase of ETP whereas Pd-MP/PS+ affected mainly ETP. However the increase of Ed-MP and Pd-MP plasma concentration does not appear to be by itself a sufficient condition to induce a significant increase of TG. Disclosures: No relevant conflicts of interest to declare.

CD32, CD18, CD11b and CD62L Expression on Monocyte without and After Lipopolysaccharide (LPS) Challenge: Association with Inflammatory and Hemolysis Markers In Sickle Cell Anemia.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1655-1655
Author(s):  
Bruno A. V. Cerqueira ◽  
Wendell Vilas-Boas ◽  
Jose Moura Neto ◽  
Jorge Clarencio ◽  
Daniela Andrade ◽  
...  
Keyword(s):  
Lipid Metabolism ◽  
Medical History ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
Control Group ◽  
Surface Molecule ◽  
Hemoglobin S ◽  
Lps Challenge ◽  
Hematological Analysis

Abstract Abstract 1655 Introduction: Vaso-occlusive episodes (VOE) in sickle cell anemia, homozygous form of hemoglobin S (HB S), involve interactions between sickle red blood cells (RBC), endothelial cells, leukocytes, platelets, coagulation factors and plasma proteins. The purpose of the present study was explore the inflammatory potential of monocytes, activation state, response against challenge and its association with hemolytic, inflammatory, lipid metabolism and medical history of SCA patients. Patients and Methods: We studied 27 SCA patients in steady-state (11 men, 16 women, mean age: 22.26 ± 16.03 years) from Northeast Brazil diagnosed with SCA in attendance of the outpatients clinic of the Foundation of Hematology and Hemotherapy of Bahia (HEMOBA). The control group was compound by 20 healthy Brazilian, with AA hemoglobin pattern matched by age, years and ethnic origin. The study was approved by the FIOCRUZ ethical committee and informed consents were signed by patients or official responsible. The lipid and hemolytic profile were measured by biochemical colorimetric methods, hematological analysis and hemoglobin profile were performed using an electronic cell counter and HPLC, respectively, cytokines and surface molecules expressions on monocytes were evaluated by flow cytometry (labeled with monoclonal anti-CD11b, anti-CD18, ant-CD32, anti-CD62L and anti-HumanTNF), the monocyte activation was stimulated by LPS and the complete medical history was obtained by patients' record. Results: Our results show higher CD11b monocyte expression in SCA patients than control group (p=0.000) and lower CD18 and CD32 on monocyte expression in SCA patients than control group (p=0.001; p=0.018). After LPS stimulation, we observed increased expression of CD11b, CD18 and CD32 expression on monocyte in SCA (p=0.000) so as in the control group (p=0.000). Moreover, monocyte expression of CD32 was negatively associated with aspartate aminotransferase (r=-0.468; p=0.014) and hemoglobin S (r=-0.389; p=0.045), but presented positive correlation with TNF-alpha (r=0.414; p=0.044). In another hand, the monocyte expression of CD62L was associated with an increase of hemoglobin concentration (r=0.496; p=0.009) and high density lipoprotein-cholesterol (r=0.505; p=0.007). Sickle cell anemia individuals that developed splenic sequestration exhibited increased expression of CD11b on monocyte surface. Conclusion: The study of monocyte surface molecule and its association with markers of hemolysis, inflammation and lipid metabolism may indicate a differentiated mechanism of these molecules in sickle cell anemia pathogenesis, with a complex involvement of cellular, endothelial and proinflammatory interactions. Additional studies should be carried out in order to explore the contribution of monocyte expression surface molecule on vascular occlusion in the sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.

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