scholarly journals Evaluation of Baseline Hematologic Parameters of Steady State Sickle Cell Disease Patients at The Chantal Biya’s Foundation, Yaounde- Cameroon

2019 ◽  
Vol 7 (07) ◽  
Author(s):  
Bih Adelaide ◽  
Ngala Solange Mudih ◽  
Alima Yanda ◽  
Akaba Fergus Ambe ◽  
Jutcha Florent ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Reference Values ◽  
Blood Cells ◽  
Healthy Subjects ◽  
White Blood Cells ◽  
Great Decrease ◽  
Cell Disease ◽  
Mean Values

Introduction Sickle cell disease is a genetic abnormality involving the hemoglobin. Although it is primarily a red blood cell disorder, the white blood cells and platelets are also affected by the mutation. The consequence hemoglobin S causing polymerization of hemoglobin, results in hemolysis and anemia. This study aims to provide baseline hematologic parameters in steady state sickle cell disease patients compared with the reference values of normal healthy subjects used at the Chantal Biya Foundation (CBF), in order to monitor other sicklers in Cameroon. Methodology A comparative analysis of sickle cell hematologic parameters with control hematologic parameters of normal healthy subjects (reference values of healthy subjects used at the Chantal Biya Foundation) was carried out. Results A total of 62 sickle cell disease patients in steady state who complied with the selection criteria were recruited. Of the 62, 29 were females and 33 were males. The age range was from 1 year to 19 years and an average age of 6 ± 4.19 SD. Results from sickle cell patients showed an increase in white blood cells (WBCs), neutrophils and lymphocytes and a great decrease in the mean values of hematocrit Hct. as well as RBC indices, but no great or slight difference in the values of basophils compared with the reference values of normal healthy subjects in the CBF Yaoundé, 2015. Conclusion Sickle cell disease patients in steady state have lower values of red cells parameters, but higher values of white cells and platelets count when compared with the reference values of normal healthy subjects used at the Chantal Biya Foundation.

scholarly journals The prevalence of leukocyte abnormalities among Sudanese patients with sickle cell disease

2021 ◽  
Vol 9 (1) ◽  
pp. 262-267
Author(s):  
Tarig Osman Khalafallah Ahmed ◽  
Ekhlas Alrasheid Abu Elfadul ◽  
Ahmed A. Agab Eldour ◽  
Omer Ibrahim Abdallah Mohammed
Keyword(s):  
Sickle Cell Disease ◽  
Cell Volume ◽  
Sickle Cell ◽  
Blood Cells ◽  
White Blood Cells ◽  
Blood Film ◽  
Cell Disease ◽  
Mean Cell Volume ◽  
Blood Disorder ◽  
Age Range

Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells. The study was conducted in Elobied town during the period May 2011 to September 2011. The aim of this study is to detect the abnormalities of leucocytes among sickle cell anemic patients. 40 sickle cell anemic patients; age range between 8 months to 23 years. Blood sample was taken for all patients and the laboratory investigation were performed using automated estimation for: hemoglobin (Hb), Packed cell volume (PCV), red cell count (RBCs), mean cell volume (MCV), mean cell hemoglobin (MCH), mean cell concentration (MCHC), and total white blood cells, comment on blood film using manual methods. The conclusion of this study there is increase in total white blood cells with shift to left in neutrophil precursor in sickle cell patients with complications ,the most immature cells are band form, myelocytes and metamyelocytes, and there also lymphocytosis and neutrophilia which has been increases in response to infections.

scholarly journals Alpha-1 Antitrypsin and SERPINA1 gene Mutation As New Biomarker in Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4918-4918
Author(s):  
Magda Oliveira Seixas Carvalho ◽  
Carvalho Luís André ◽  
Mauri­cio Batista Carvalho ◽  
Larissa Carneiro Rocha ◽  
Valma Maria Lopes Nascimento ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Gene Polymorphisms ◽  
Clinical History ◽  
Control Group ◽  
Cell Disease ◽  
Serpina1 Gene ◽  
Alpha 1 Antitrypsin

Abstract Sickle cell disease (SCD) has heterogeneous clinical picture and there are several pathway involved in SCD pathogenesis, and chronic inflammation and hemolysis are important hallmarks of the disease. Based on such points, there is a search for prognostic markers to establish possible sub-genotypes of the disease. The present study investigated the alpha-1 antitrypsin (AAT) levels, a serum glycoprotein inhibitor of proteases responsible for trigger inflammatory reactions, describing its associations with SERPINA1 gene polymorphisms, hematological and chemistry biomarkers, and clinical history in a group of SCD children in steady-state. The study was approved at FIOCRUZ research board and followed the principle of Declaration of Helsinki. A total of 356 steady state unrelated SCD patients were included at the present study and a control group (CG) compound by 100 unrelated healthy individuals sex and age matched with the patients group, which were from the same geographical origin. Patients age ranging 13.96+9.91 years of age, the AAT levels higher than the 50th percentile (158.0 mg/mL) had significantly lower red blood cells (RBC) count (p=0.003), hemoglobin (Hb) (p=0.0002) and hematocrit (Hct) (0.0002) concentration, and higher white blood cells (WBC) (p=0.004) and neutrophils (p=0.0001) counts, and higher C-reactive protein (CRP) levels (p<0.0001), and lower urea levels (p=0.0003). AAT had significant negative correlation with RBC (r=-0.205, p=0.0001), Hb (r=-0.203, p=0.0001), Hct (r=-0.256, p<0.0001), high density lipoprotein cholesterol (HDL-C) (r=-0.205, p=0.0001), urea (r=-0.184, p=0.0005), creatinine (r=-134, p=0.012), and albumin (r=-0.135, p=0.011), and significant positive correlation with WBC (r=0.18, 0.0007), neutrophils (r=0.2297, p<0.0001), HbS (r=0.2874), total bilirubin (r=0.137, p=0.01), direct bilirubin (r=0.136, p=0.001), indirect bilirubin (r=0.115, p=0.032), lactate dehydrogenase (r=0.159, p=0.003), ferritin (r=0.1353, 9=0.011), CRP (r=0.355, p<0.0001). Patients with higher levels of AAT had clinical history of more than three infection episodes (OR=1.71, CI:1.05-2.65, p=0.02), gallstones presence (OR=1.75, CI:1.03-2.97, p=0.02), and received more blood therapy (OR=2.35, CI:1.51-3.65, p=0.0001).The SERPINA1 gene polymorphisms were analyzed in 126 SCD patients and 100 CG individuals, the protease inhibitor (Pi)*MM or wide type genotype was find in 115 (91.3%) of SCD patients and 92 (92%) of CG; the PI*SS genotype was find in 2 (1.6%) SCD patients and in none individual of the CG; the PI*MS genotype was find in 9 (7.1%) SCD patients and in 6 (6%) CG individuals; and the PI*MZ genotype was find in 2 (2%) CG individuals. Ours results suggest that the AAT may be considerer as a marker of SCD patients’ outcome, once increased levels is related to stress situation, anti-inflammatory and inhibitory proteases properties and may act as a physiological and cytoprotective regulator influencing the severity of SCD. Our results related to SERPINA1 genotypes emphasize the role of the mutant allele in decrease the AAT production what may represent a risk factor. Additional studies should be carried out in order to investigate mechanisms throughout the AAT can induce several important events in the SCD pathogenesis. Disclosures No relevant conflicts of interest to declare.

The Use Of Two Photon Microscopy To Image Vaso-Occulsion In Pulmonary Microvessels Of Living Mice With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 976-976
Author(s):  
Natacha Ralainirina ◽  
Ferron Lynn Sonderegger ◽  
Hong Pei ◽  
Grzegorz Chodaczek ◽  
Joel Linden
Keyword(s):  
Endothelial Cells ◽  
Sickle Cell Disease ◽  
Real Time ◽  
Sickle Cell ◽  
Intravital Microscopy ◽  
Blood Cells ◽  
White Blood Cells ◽  
Cell Disease ◽  
Alexa Fluor ◽  
Two Photon

Abstract Although sickle cell anemia is initiated by red cell pathology, it is accompanied by an inflammatory immune response involving platelets and white blood cells that contribute to vaso-occlusvie episodes including painful vaso-occlusive crises (pVOC) and acute chest syndrome (ACS). In order to better understand the cellular and molecular bases of vaso-occlusion we are in the process of developing procedures to image microvessels in the lung, liver and spleen of living mice by 2-photon microscopy, a procedure that is based on excitation of a fluorophore by two photons simultaneously. The two-photon technique utilizes infrared light that efficiently penetrates tissues up to 200 microns with low phototoxicity allowing time-lapse imaging. Two-photon intravital microscopy can be used to study the behavior of intravascular cells during vaso-occlusive events. Mice are prepared for lung intravital microscopy by the intraperitoneal injection of a mixture of ketamine and xylazine. Additional anesthesia is added during experimentation. The trachea is opened and the mouse intubated. The chest is opened to allow access to the left lobe of the lung through a window that is a few millimeters in diameter. PBS is applied to keep the lung moist. A custom built suction device is placed on the lung and covered with a cover glass at the same time pressure is exerted to seal the organ and the glass cover together. Throughout the procedure, the mouse is held at a temperature of 37°C. Once surgery is completed, a mixture of antibodies coupled to fluorophores is given by retro-orbital injection. In order to minimize photobleaching we used antibodies conjugated to Alexa Fluor 488, Alexa Fluor 555 or Alexa Fluor 647. We are able to visualize and quantify interactions between red blood cells, white blood cells, and endothelial cells as well as the expression of adhesion molecules on endothelial cells in real time. During pVOC triggered by hypoxia, cell adhesion of neutrophils, lymphocytes and monocytes to the endothelium is observed that is associated with an increase in endothelial expression of ICAM-1 and V-CAM. We label endothelial cells with anti-CD31, lymphocytes with anti-CD45, monocytes with anti-Ly6C and neutrophils with anti-Ly6G. Platelets are labeled with anti-CD41 or anti-CD62P, NK cells with anti-NKp46, and macrophages with anti-F4/80 and anti-CD1d. We are able to quantify cell shape, rolling, adhesion and movement. Our preliminary results demonstrate that it is possible in real time to image the sequence of events occurring during pulmonary vasoocclusion in sickle cell disease. In conclusion, intravital 2-photon microscopy holds great potential for enabling us to better understand inflammatory responses within the blood vessels of living SCD mice. Disclosures: No relevant conflicts of interest to declare.

scholarly journals High-Throughput Mirna Analysis Suggests Pro-Inflammatory Profile in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1077-1077
Author(s):  
Matthew Cannon ◽  
Sarah Glass ◽  
Sidney Smith ◽  
Melanie Heinlein ◽  
Rosa Lapalombella ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Research Funding ◽  
Red Cell ◽  
Cell Disease ◽  
Acute Crisis

Abstract BACKGROUND: Mature circulating red blood cells, though devoid of a nucleus, have been shown to contain an abundance of miRNAs. Further, it has been shown that sickle cell patient-derived RBCs have a dramatic difference in miRNA content than normal RBCs. Given that a range of miRNAs are involved in the regulation of immunity, including the release of inflammatory mediators, we hypothesize that miRNAs enriched in circulating red blood cells function to prolong the inflammatory state in sickle cell disease. Further, we hypothesize that these miRNAs can be used as biomarkers for use in the clinic to predict crisis and differentiate acute versus chronic pain. Exploring this miRNA enrichment in circulating red blood cells in sickle cell patients will provide practical insight for the inflammation state and will inform characteristics of patients who may need greater care in the clinic. METHODS: Twenty steady state patients were recruited and categorized according to their chronic pain status and crisis frequency per year. Whole blood was drawn during routine visits to the OSU Wexner Medical Center Hematology Clinic. Additionally, whole blood was drawn from five patients either in acute pain crisis (recruited prior to crisis) or within a few days of crisis. Samples were subject to double gradient centrifugation and red cells were resuspended in Trizol and cryopreserved. MiRNAs were isolated from red cell Trizol suspensions using a commercial isolation kit (QIAGEN Cat#217004). Isolated miRNAs were then subject to a NanoString Human miR (v3) expression assay. Differential expression analysis was conducted to compare miRNAs with at least 1.5 fold difference (p = 0.05) between steady state and acute crisis. Target prediction and GO ontology analysis was performed for statistically significant miRNAs using DIANA Tools mirPath v3. Follow-up qPCRs were performed using TaqMan Advanced miRNA cDNA Synthesis Kit (Cat#A28007) and TaqMan Advanced miRNA Assays (Cat#A25576) to validate the decreased expression of miRNAs. Additional qPCRs were performed using TaqMan Gene Expression Assays (Cat#4331182) to investigate mRNA regulatory effects of significant miRNAs in the total red cell population. Western blots were also performed to investigate regulatory effects of these miRNAs at the protein level. RESULTS & CONCLUSION: Comparison of RBC miRNA profiles from patients during acute crisis to those in steady state shows several significantly decreased (>1.5 fold) miRNAs in crisis. Among these miRs we have found previously uncharacterized miRNAs, hsa-miR-2116-5p and hsa-miR-302d-3p. DIANA tools miRNA analysis software predicts these miRNAs to be involved in regulation of cell-to-cell adhesion pathways through gene transcripts such as Protocadherin Beta 6 (PCDHB6) and Neural Cell Adhesion Molecule 2 (NCAM2). Interestingly, inspection of miRNA predicted targets that fall under significant GO terms also predicts several individual miRNAs to regulate inflammatory response and nociceptive signaling gene transcripts like A20 (TNFAIP3) and Cathepsin S (CTSS). Validation of these miRNAs was performed via qPCR for 5 out of the 6 significantly decreased miRNAs. Of the 5 miRNAs tested, hsa-miR-2116-5p, hsa-miR-302d-3p, and hsa-miR-1246 were validated as having decreased expression in acute crisis patients compared to steady state. qPCRs were then performed to probe for miRNA based regulation of top predicted target mRNA transcripts. Both CTSS and TNFAIP3 showed increased expression of mRNA transcripts in acute crisis patient red cells as compared to steady state. Next, western blot analysis was performed on red cell protein lysate. Interestingly, this analysis revealed a pattern in activated CTSS expression that was independent of acute crisis. Steady state patients reporting chronic pain showed increased activated CTSS compared to those without chronic pain. Activated CTSS was not found in red cell lysates from three normal, non-SCD donors. Taken together, these results suggest that red blood cells may play a larger role in inflammation and pain responses in sickle cell disease than previously thought. Further these results suggest activated CTSS as a potential biomarker for differentiating chronic pain in patients. Follow-up studies are underway to further stratify and investigate these findings. Disclosures Desai: University of Pittsburgh: Research Funding; Ironwood: Other: Adjudication Committee; NIH: Research Funding; FDA: Research Funding; Selexy/Novartis: Research Funding; Pfizer: Research Funding.

scholarly journals Management of Hodgkin Lymphoma in a Sickle Cell Patient: A Case Report

2019 ◽  
Vol 12 (1) ◽  
pp. 224-227
Author(s):  
Farah Ashraf ◽  
Pragnan Kancharla ◽  
Mendel Goldfinger
Keyword(s):  
Quality Of Life ◽  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Patient Population ◽  
White Blood Cells ◽  
Modern Medicine ◽  
Cell Disease

Sickle cell disease (SCD) is an inherited disorder of hemoglobin mutation in red blood cells, with a patient population that is increasing in age in recent decades due to advances in modern medicine. Hodgkin’s lymphoma (HL) is a cancer of white blood cells, and while concomitance of SCD and Hodgkin’s has been reported, a discussion of treatment for HL in SCD is lacking from the literature. We present a case of effectively treated HL in SCD and put forth that the regimen used is a practical choice, and as it was completed fully as outpatient, it improved the patient’s quality of life compared to an inpatient regimen.

scholarly journals Elevated Urinary Leukotriene E4 Levels Are Associated with Hospitalization for Pain in Children with Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3405-3405
Author(s):  
Jeanine E. Jennings ◽  
Thiruvamoor Ramkumar ◽  
Jingnan Mao ◽  
Jessica Boyd ◽  
Mario Castro ◽  
...  
Keyword(s):  
Arachidonic Acid ◽  
Sickle Cell Disease ◽  
Incidence Rate ◽  
Sickle Cell ◽  
Blood Cells ◽  
Medical Records ◽  
White Blood Cells ◽  
Cell Disease ◽  
Inflammatory Stimuli ◽  
Inflammatory State

Abstract Introduction: Sickle cell disease (SCD) is associated with an inflammatory state. Luekotrienes are inflammatory mediators derived from arachidonic acid and produced by white blood cells in response to inflammatory stimuli. We tested two hypotheses among children with SCD: Baseline leukotriene levels are elevated in SCD when compared to controls children without SCD, Baseline leukotriene levels are associated with an increased incidence rate of hospitalization for pain. Methods: During routine clinical visits, baseline urinary leukotriene E4 (LTE4) levels were measured in children with SCD (cases) and age, and ethnic, matched children without SCD (controls). Medical records of the cases were reviewed to assess the frequency of hospitalization for pain within three years of entering the study. Results: LTE4 levels were obtained in 71 cases and 22 controls. LTE4 levels were higher in the cases compared to controls (median LTE4: 100 vs.57 pg/mg creatinine); P < 0.001. After adjustment for age and diagnosis of asthma, a greater incidence rate of hospitalization for pain was observed among children with SCD in the highest tertile group when compared to the lowest tertile group (114 vs. 52 episodes per 100 patient-years, P=0.038). Conclusion: LTE4 levels are elevated in children with SCD when compared to controls without SCD and are associated with an increased rate of hospitalizations for pain.

Myeloid Dysfunction in Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4908-4908
Author(s):  
Alessandra Romano ◽  
Nunziatina Laura Parrinello ◽  
Concetta Conticello ◽  
Rossellina Rosso ◽  
Perrotta Caterina ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Peripheral Blood ◽  
Healthy Subjects ◽  
Lymphoid Cells ◽  
Cell Disease ◽  
Myeloid Suppressor Cells ◽  
Hla Dr ◽  
Organ Systems

Abstract Background In sickle cell disease (SCD), profound anaemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems, including immune system. Infectious complications are common, constitute the second most common cause of mortality and a main cause of morbidity. During the haemolytic crisis, large amount of arginase (s-Arg-1) are released, potentially acting as immunosuppressor molecule. Despite its clinical impact, only a few is known about myeloid dysregulation in SCD. Aim Detecting immunological impairment at the steady state evaluating myeloid and lymphoid cells in peripheral blood of SCD patients. Material and Methods Between May and June 2014,peripheral blood obtained from 30 consecutive SCD patients at the steady state plus 30 healthy subjects was studied for evaluation of myeloid subpopulations and lymphoid paresis. Myeloiddys function was evaluated as percentage and absolute count of circulating myeloid suppressor cells (MDSC) in peripheral blood assessed by flow cytometry as follows: im-MDSC (CD34+/CD11b+/CD13+/CD14-/ HLA-DR-/CD45+), neutrophilic-like N-MDSC (CD11b+/CD13+/CD15+/CD14-/HLA-DR-/Lin-) and monocytic-likemo-MDSC (CD14+/HLA-DRlow/-), phagocytic activityof granulocytes using a commercially available kit (Phagotest R), amount of Arg-1 expressed in mature granulocytes by RT-PCR and circulating s-Arg-1 using a commercially available ELISA kit (Biovendor). Results The capability of phagocytosis of granulocytes wassignificantly reduced compared to healthy subjects (p=0.001). G-MDSC subset was not increased, while mo-MDSC subpopulation was increased in SCD (p=0.001) but not in thalasso-SCD. s-ARG-1 was increased in both SCD and thalasso-SCD (respectively 203±3 ng/mL and 248±6ng/mL, p=0.003) and positively correlated with the amount of HbS (r=0.7, p=0.002). Arg-1 expression in granulocytes was increased (20 times higher than healthy controls, p=0.002) Conclusion SCD and thalasso-SCD caucasian patients exhibiti mmunosuppressive myeloid markers including reduced phagocytosis, increased amount of mo-MDSC, Arg-1 expression in granulocytes and circulating s-Arg-1. Further analysis are ongoing to detect if the same myeloid impairment occurs during vase occlusive crisis and in a different genetic background, like in Afro-Americans. Disclosures No relevant conflicts of interest to declare.

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