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

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.

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

Blood ◽

10.1182/blood.v114.22.4628.4628 ◽

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.

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

Blood ◽

10.1182/blood.v124.21.1390.1390 ◽

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.

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MBL2 gene polymorphisms are not related to the occurrence of cerebrovascular disease in sickle cell anemia

Research Society and Development ◽

10.33448/rsd-v9i7.4240 ◽

2020 ◽

Vol 9 (7) ◽

pp. e439974240

Author(s):

Isabela Cristina Cordeiro Farias ◽

Taciana Furtado Mendonça-Belmont ◽

Patrícia Muniz Mendes Freire Moura ◽

Igor Farias Domingos ◽

Diego Arruda Falcão ◽

...

Keyword(s):

Cerebrovascular Disease ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Promoter Region ◽

Gene Polymorphisms ◽

Control Group ◽

Mbl2 Gene ◽

Number Of Patients ◽

Exon 1 ◽

The Impact

Objective: This study has as objective to verify whether MBL2 gene polymorphisms are related to the occurrence of cerebrovascular disease (CD) in sickle cell anemia (SCA) patients. Methods: Overall, 259 unrelated SCA patients were enrolled. The patients were divided into three groups: control group, stroke group ad range of risk group. Peripheral blood samples were collected and DNA extraction was performed. All patients were genotyped for exon 1, promoter region -221 and promoter region -550 of MBL2 gene, along with β-globin gene haplotypes. Results: Concerning the genotyping of the MBL2, there was no difference in the frequency of allelic and genotypic variants of the exon 1 and the promoter regions -221 and -550 of the MBL2 gene among the studied groups. Conclusion: Despite the small number of patients, and the lack of association between MBL2 polymorphisms and CD, our study represents an effort to understand the impact of MBL2 polymorphisms in the clinical outcome of patients with SCA.

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Assessment of Salivary Zink Level and its impact on Periodontal Health among Children and Adolescence with Sickle Cell Anemia

10.21203/rs.3.rs-821539/v1 ◽

2021 ◽

Author(s):

Rogyia Eltayeb Hassan ◽

Lamis AbdelGadir Kaddam

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Serum Zinc ◽

Zinc Level ◽

Control Group ◽

P Value ◽

Periodontal Health ◽

Low Level ◽

The Mean ◽

The Impact

Abstract Background: Sickle cell anemia (SCA) is the most common inherited disorder around the world. It affects red blood cells resulting in severe hemolysis and painful crisis with multi-organ damage. Sickle cell anemia patients showed an impaired antioxidant status due to a reduced antioxidant defense mechanism that depends indirectly on zinc micronutrient. Several studies revealed a low level of serum zinc among sickle patients. However, none of them investigates the impact of SCA on salivary zinc level. This study aims to measure zinc level in saliva and its influence on the periodontal health of Sudanese children with SCA in Khartoum state. Up to our knowledge, this the first study conducted to measure salivary Zink level and its impact on periodontal health among sickle patients Subjects and Methods: Cross-sectional study was conducted among children and adolescents aged 5-18 years old. Sixty individuals were involved, 30 with SCA compared to 30 healthy controls groups. An Atomic Absorption spectrophotometer quantified the salivary level of zinc. Periodontal examinations were assessed in SCA children using the periodontal community index.Results: The mean ages in the sickle cell anemic group were 10.37 ± 4.13 years old, while the mean ages of subjects in the control group were 9.37 ± 3.6 years (P.Value: 0.169). Salivary zinc level was significantly lower among SCA patients (P.Value <0.001) than in the healthy control group. None of the participants were diagnosed with periodontitis. The less salivary zinc level was significantly higher in SCA with standard pocket depth and no clinical attachment loss than patients diagnosed with gingivitis ( P. Value:0.025).Conclusions: Sickle patients have a low level of zinc in saliva, which may contribute to some manifestations of sickle cell disease and may make them more susceptible to periodontal disease in the future. We recommend conducting more research on oral and periodontal health among children affected with SCA to improve their quality of life.

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Transcranial Doppler Screening in Children with Sickle Cell Anemia Is Feasible in Central India and Reveals High Risk of Stroke

Blood ◽

10.1182/blood-2019-131416 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2279-2279

Author(s):

Dipty Jain ◽

Kavitha Ganesan ◽

Sati Sahota ◽

Deepika S. Darbari ◽

Lakshmanan Krishnamurti ◽

...

Keyword(s):

High Risk ◽

Oxygen Saturation ◽

Board Of Directors ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Transcranial Doppler ◽

Central India ◽

Normal Range ◽

Advisory Committees ◽

Tribal Populations

Introduction: India has been identified as having the second largest number of births with sickle cell anemia (SCA) in the world after Africa, with estimated 44,400 new-borns affected per year. SCD was previously reported to have a milder course in children from India, with less severe disease among aboriginal tribal populations than in non-tribal populations. Recent reports indicate the occurrence of severe manifestations of SCD in both tribal and non-tribal populations in India. Stroke is one of the serious complications of SCD, but there are no data on transcranial Doppler (TCD) screening for evaluating children with SCD in India who may be at high risk for strokes. The objective of this study was to assess the feasibility of using TCD to measure time averaged maximum of the mean velocities (TAMMV) in the intracranial arteries in children attending a tertiary centre in central India. Methods: STUDY DESIGN: A cross sectional study was conducted in consecutively recruited stable children of either sex with homozygous SCA proven by electrophoresis and high performance liquid chromatography in the age group of 1-26 years. Patients who were febrile, acutely ill, hypoxic or asleep were not included in the study as these conditions can falsely elevate the intracranial blood flow velocities. Patients with hemoglobinopathies other than HbSS or S/b0 Thalassemia and those with a history of congenital neurological illness were excluded. DETERMINATION OF TCD VELOCITY: TCD was performed in a tertiary care center in Nagpur using either an imaging machine (Lasiq s8) in the department of radiology or a portable non-imaging TCD (Compumedics); for both a probe of frequency 2Mhz was used. Maximum values for TAMMV in the Middle (MCA) and Anterior (ACA) cerebral arteries were measured in all; for the non-imaging TCD values for posterior cerebral artery (PCA) and basilar artery were also obtained. The results of the first scan performed on these individuals were included in this study. Using values similar to the STOP trial, TAMMV of each of these vessels were categorized as follows: Normal <= 170cm/s; Conditional - between 170 and 199 cm/s; Abnormal >= 200 cm/s; Low <50 cm/s and unobtainable. MEASUREMENT OF HAEMATOLOGICAL VALUES: Laboratory parameters such as Hemoglobin, white blood cell count (WBC), Mean corpuscular volume (MCV) and hemoglobin F (HbF) levels of the patients in the study were also included if the parameters were available on the day of TCD or within 90 days of TCD study. MEASUREMENT OF HEIGHT, WEIGHT AND BMI: The height and weight of each of the patients on the day of TCD or within a period of 60 days from the TCD were measured and the body mass index (BMI) was calculated. Results One hundred and twenty children and youth aged 1-26 (median 7) years, 67 male (56%), were recruited. Of the 120 patients, 106 (88.5%) belonged to the Scheduled Caste category, 3 (2.5%) to the Scheduled Tribe category and 11 (9.1%) to the Other Classes category. Three (2.5%) had had a clinical stroke and 8 (7%) had had seizures, one of whom also had a stroke. Twenty-seven (23%) children had TAMMV outside the normal range. Five had abnormal TAMMV in the MCA (n=4) and/or ACA (n=1), 8 had conditional TAMMV in the MCA (n=7) and/or ACA (n=1) while 14 patients had low (n=12) or unobtainable (n=2) TAMMV in the MCA. One child with stroke had low TAMMV and one had conditional TAMMV while the third had normal TAMMV. Of the 7 with isolated seizures, one had low TAMMV and one had conditional TAMMV while the remaining 5 were normal. BMI was 8.6-25.3 (median 14.1), height/weight was 3.4-10.3 (median 6.5), hemoglobin was 43-134 (median 81) g/L, oxygen saturation 87-100 (median 99)%, HbF was 1.9-60 (median 21) g/dL, MCV was 59.1-96.7 (median 83.2) fl, WBC was 2.3-35.9 (median 10.1)*109. Those with TAMMV outside the normal range were not different from those with normal TAMMV in terms of age, BMI, Height/weight, or recent hemoglobin, oxygen saturation, HbF, MCV, or WBC. Discussion This study demonstrates the feasibility and importance of TCD screening in Indian SCD population. TAMMV on TCD was outside the normal range in nearly a quarter of children with SCD, as has been reported in studies in other populations. The findings of this study may not be representative of stroke risk in tribal populations since they were underrepresented in this study. These data provide the rationale for implementing systematic screening with TCD to reduce the risk of stroke in children affected by SCD in India. Disclosures Darbari: Novartis: Membership on an entity's Board of Directors or advisory committees; Hilton Publishing: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Other: one day advisory board meeting .

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Reticulocyte count is the most important predictor of acute cerebral ischemia and high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia

Annals of Hematology ◽

10.1007/s00277-016-2789-5 ◽

2016 ◽

Vol 95 (11) ◽

pp. 1869-1880 ◽

Cited By ~ 7

Author(s):

André Rolim Belisário ◽

Rahyssa Rodrigues Sales ◽

Nayara Evelin Toledo ◽

Maristela Braga de Sousa Rodrigues Muniz ◽

Cibele Velloso-Rodrigues ◽

...

Keyword(s):

Cerebral Ischemia ◽

High Risk ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Transcranial Doppler ◽

Reticulocyte Count ◽

Important Predictor ◽

Acute Cerebral Ischemia

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Deferasirox for the Treatment of Transfusional Iron Overload in Sickle Cell Anemia. Preliminary Results

Blood ◽

10.1182/blood.v112.11.2879.2879 ◽

2008 ◽

Vol 112 (11) ◽

pp. 2879-2879

Author(s):

Rodolfo D Cancado ◽

Maria Cristina A Olivato ◽

Paula Bruniera ◽

Carlos Chiattone

Keyword(s):

Serum Creatinine ◽

Iron Overload ◽

Sickle Cell ◽

Serum Ferritin ◽

Sickle Cell Anemia ◽

The Body ◽

Once Daily ◽

Body Iron ◽

Endocrine Organs ◽

The Impact

Abstract The majority of patients with sickle cell anaemia have received repeated blood transfusions by adulthood. Because the body has no physiological mechanism to actively excrete the excess of iron, chelation therapy is important for the management of iron overload and its complications, including iron deposition into the liver, heart and endocrine organs, eventual death. While studies are limited, progressive iron loading and subsequent tissue injury in sickle cell disease appears similar to other transfused populations. Deferasirox (Exjade, ICL670) is a once-daily, oral iron chelator that is approved for the first-line treatment of chronic transfusional iron overload. Its safety, tolerability and efficacy in reducing body iron burden have been demonstrated in patients with β-thalassaemia major and in other chronic transfusion-dependent anaemias. The objectives of this prospective, non-randomised, phase IV trial were to evaluate the iron overload status, before and after one year-treatment with deferasirox, using liver iron concentration (LIC) by MRI of the liver, MRI cardiac (Cardiac T2*), serum ferritin and the impact of deferasirox treatment on these measurements, and to evaluate the safety and tolerability of this drug. A total of 30 patients with sickle cell anemia and iron overload, defined as the use of ≥ 20 units of RBC units and/or two plasma ferritin levels ≥ 1000 mcg/L during the 6 months preceding enrollment, received starting dose of 20mg/kg/day of deferasirox. Efficacy was assessed monthly by measuring change from baseline in serum ferritin levels. Safety was evaluated on a monthly basis according to the incidence and type of adverse events and measurement of laboratory parameters, including serum creatinine and liver enzyme levels. Mean (range) age 26.4 ± 12.3y (9–49), 83% female, 93% afrodescendent, 60% on regular blood transfusion, mean deferasirox exposure 30.1 ± 5.6 weeks (16–39), mean MRI hepatic (LIC, μmol/g) 233.0 ± 98.8 (45 – 350), mean MRI cardiac (Cardiac T2*, ms) 41.20 ± 5.46 (27.52 – 51.19). Median ± SD and mean (range) serum ferritin level (mcg/L) at baseline and 6 months varied from 2315.5 ± 1083.9 to 2062.5 ± 1320.8 (p=0.032) and 2012.0 (1013–6074) to 1654.0 (688–6729), respectively. The proportion of patients with serum ferritin levels < 2000, 2000- <3000 and ≥ 3000 mcg/L from baseline to 6 months by percentage of patients changed from 50% to 60%, 26.7% to 26.7% and 23.3% to 13.3%, respectively. The most common drug-related AEs were mild, transient diarrhea (23.3%), headache (20.0%) and nausea (16.7%). Maculo-papular skin rash and serum creatinine increases upper limit of normal were observed in 2 (6.7%) patients. No patient experienced progressive increases in serum creatinine or renal failure. Our preliminary data, over 6-month-period of treatment, confirms that deferasirox is effective and generally well tolerated in pediatric and adult patients, and appears to have similar efficacy to deferoxamine in reducing body iron burden in transfused patients with sickle cell anemia. The availability of deferasirox as a once-daily, oral alternative would potentially facilitate improved compliance, and thereby reduce morbidity and mortality from iron overload.

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

Blood ◽

10.1182/blood.v116.21.4818.4818 ◽

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.

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

Blood ◽

10.1182/blood.v122.21.4681.4681 ◽

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.

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