scholarly journals A 24-Year-Old Woman First Diagnosed as a Sickle Cell Anemia during her Second Pregnancy: A Case Report

Author(s):  
Fatemeh Mojallal Najar ◽  
Mohammad Reza Kazemi ◽  
Ali Raee Ezzabadi

Introduction: Sickle cell anemia (SCA) is generally diagnosed in the early childhood and the disease is usually diagnosed in the first years of life by its clinical manifestations. The crises of this disease worsens during pregnancy. In this report, a 24-year-old pregnant woman with gestational age of 29 weeks and six days, G2P2A0L1, is presented. She complained of severe back and lower extremity pain. Considering the refractory pain, severe anemia, and absence of delivery process signs, further tests detected SCA for her. Preterm termination of pregnancy were performed by cesarean section regarding severe non-controlled pain, severe IUGR (intrauterine growth retardation), severe oligohydramnios, and previous caesarian section. A female baby was born with Apgar score of 8 and a birth weight of 1250 grams. Considering the high probability of vascular occlusion crisis and severe anemia associated with SCA during pregnancy, it is reasonable to seek sickle cells in all the pregnant patients with severe unexplained anemia or pain.

1993 ◽  
Vol 264 (2) ◽  
pp. C251-C270 ◽  
Author(s):  
C. H. Joiner

Cellular dehydration is one of several pathological features of the sickle cell. Cation depletion is quite severe in certain populations of sickle cells and contributes to the rheological dysfunction that is the root cause of vascular occlusion in this disease. The mechanism of dehydration of sickle cells in vivo has not been ascertained, but three transport pathways may play important roles in this process. These include the deoxygenation-induced pathway that permits passive K+ loss and entry of Na+ and Ca2+; the K(+)-Cl- cotransport pathway, activated by acidification or cell swelling; and the Ca(2+)-activated K+ channel, or Gardos pathway, presumably activated by deoxygenation-induced Ca2+ influx. Recent evidence suggests that these pathways may interact in vivo. Heterogeneity exists among sickle cells as to the rate at which they become dense, suggesting that other factors may affect the activity or interactions of these pathways. Understanding the mechanism of dehydration of sickle cells may provide opportunities for pharmacological manipulation of cell volume to mitigate some of the symptoms of sickle cell disease.


2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Amged Hussein Abdelrhman ◽  
Abdelgadir Ahmed Abdelgadir

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.


Electronics ◽  
2020 ◽  
Vol 9 (3) ◽  
pp. 427 ◽  
Author(s):  
Laith Alzubaidi ◽  
Mohammed A. Fadhel ◽  
Omran Al-Shamma ◽  
Jinglan Zhang ◽  
Ye Duan

Sickle cell anemia, which is also called sickle cell disease (SCD), is a hematological disorder that causes occlusion in blood vessels, leading to hurtful episodes and even death. The key function of red blood cells (erythrocytes) is to supply all the parts of the human body with oxygen. Red blood cells (RBCs) form a crescent or sickle shape when sickle cell anemia affects them. This abnormal shape makes it difficult for sickle cells to move through the bloodstream, hence decreasing the oxygen flow. The precise classification of RBCs is the first step toward accurate diagnosis, which aids in evaluating the danger level of sickle cell anemia. The manual classification methods of erythrocytes require immense time, and it is possible that errors may be made throughout the classification stage. Traditional computer-aided techniques, which have been employed for erythrocyte classification, are based on handcrafted features techniques, and their performance relies on the selected features. They also are very sensitive to different sizes, colors, and complex shapes. However, microscopy images of erythrocytes are very complex in shape with different sizes. To this end, this research proposes lightweight deep learning models that classify the erythrocytes into three classes: circular (normal), elongated (sickle cells), and other blood content. These models are different in the number of layers and learnable filters. The available datasets of red blood cells with sickle cell disease are very small for training deep learning models. Therefore, addressing the lack of training data is the main aim of this paper. To tackle this issue and optimize the performance, the transfer learning technique is utilized. Transfer learning does not significantly affect performance on medical image tasks when the source domain is completely different from the target domain. In some cases, it can degrade the performance. Hence, we have applied the same domain transfer learning, unlike other methods that used the ImageNet dataset for transfer learning. To minimize the overfitting effect, we have utilized several data augmentation techniques. Our model obtained state-of-the-art performance and outperformed the latest methods by achieving an accuracy of 99.54% with our model and 99.98% with our model plus a multiclass SVM classifier on the erythrocytesIDB dataset and 98.87% on the collected dataset.


Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 426-432 ◽  
Author(s):  
Joshua J. Field

AbstractRecent insights into the pathogenesis of microvascular occlusion downstream of the sickled red cell have revealed new therapeutic targets for sickle cell disease (SCD). After the formation of sickle cells, tissue injury spurs inflammation, which leads to receptor-mediated contacts between sickle cells, leukocytes, and vascular endothelium. Specifically, selectins decelerate sickled red cells and leukocytes in the circulation to facilitate endothelial adhesion and other cell–cell interactions, ultimately leading to vascular occlusion. Invariant NKT (iNKT) cells, activated during reperfusion, generate a broad inflammatory response, which further increases cellular adhesion and vascular occlusion. Novel therapies are in development that target selectins and iNKT cells to prevent or interrupt the vicious cycle of adhesion and inflammation. Although the therapies hold promise for the treatment of SCD, an underappreciated threat to their development is poor access to care for people with SCD. Unless the majority of people with SCD have access to consistent, high-quality care, they will not have the opportunity to participate in a clinical trial or receive any new therapy, regardless of its efficacy.


Anemia ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Zhou Zhou ◽  
Molly Behymer ◽  
Prasenjit Guchhait

Sickle cell anemia (SCA) is a common hemolytic disorder caused by a gene mutation in theβ-globin subunit of hemoglobin (Hb) and affects millions of people. The intravascular hemolysis releases excessive amount of extracellular hemoglobin (ECHb) into plasma that causes many cellular dysfunctions in patients with SCA. ECHb scavenges NO which promotes crisis events such as vasoconstriction, thrombosis and hypercoagulation. ECHb and its degradation product, heme, are known to cause oxidative damage to the vessel wall and stimulate the expression of adhesive protein ligands on vascular endothelium. Our study shows that ECHb binds potently to VWF—largest multimeric glycoprotein in circulation—through the A2-domain, and significantly inhibits its cleavage by the metalloprotease ADAMTS13. Furthermore, a subpopulation of VWF multimers bound to ECHb exists in significant amount, accounting for about 14% of total plasma VWF, in SCD patients. The Hb-bound VWF multimers are resistant to ADAMTS13, and are hyperactive in aggregating platelets. Thus, the data suggest that Hb-bound VWF multimers are ultralarge and hyperactive because they are resistant to the protease. The Hb-bound VWF multimers are elevated parallely with the level of ECHb in patients' plasma, and is associated with the pathogenesis of thrombosis and vascular occlusion in SCA.


Blood ◽  
2010 ◽  
Vol 115 (2) ◽  
pp. 215-220 ◽  
Author(s):  
Julie Makani ◽  
Albert N. Komba ◽  
Sharon E. Cox ◽  
Julie Oruo ◽  
Khadija Mwamtemi ◽  
...  

Abstract Approximately 280 000 children are born with sickle cell anemia (SCA) in Africa annually, yet few survive beyond childhood. Falciparum malaria is considered a significant cause of this mortality. We conducted a 5-year prospective surveillance study for malaria parasitemia, clinical malaria, and severe malarial anemia (SMA) in Dar-es-Salaam, Tanzania, between 2004 and 2009. We recorded 10 491 visits to the outpatient clinic among 1808 patients with SCA and 773 visits among 679 patients without SCA. Similarly, we recorded 691 hospital admissions among 497 patients with SCA and 2017 in patients without SCA. Overall, the prevalence of parasitemia was lower in patients with SCA than in patients without SCA both at clinic (0.7% vs 1.6%; OR, 0.53; 95% CI, 0.32-0.86; P = .008) and during hospitalization (3.0% vs 5.6%; OR, 0.46; 95% CI, 0.25-0.94; P = .01). Furthermore, patients with SCA had higher rates of malaria during hospitalization than at clinic, the ORs being 4.29 (95% CI, 2.63-7.01; P < .001) for parasitemia, 17.66 (95% CI, 5.92-52.71; P < .001) for clinical malaria, and 21.11 (95% CI, 8.46-52.67; P < .001) for SMA. Although malaria was rare among patients with SCA, parasitemia during hospitalization was associated with both severe anemia and death. Effective treatment for malaria during severe illness episodes and further studies to determine the role chemoprophylaxis are required.


Blood ◽  
2001 ◽  
Vol 98 (5) ◽  
pp. 1577-1584 ◽  
Author(s):  
Kitty de Jong ◽  
Renee K. Emerson ◽  
James Butler ◽  
Jacob Bastacky ◽  
Narla Mohandas ◽  
...  

Several transgenic murine models for sickle cell anemia have been developed that closely reproduce the biochemical and physiological disorders in the human disease. A comprehensive characterization is described of hematologic parameters of mature red blood cells, reticulocytes, and red cell precursors in the bone marrow and spleen of a murine sickle cell model in which erythroid cells expressed exclusively human α, γ, and βS globin. Red cell survival was dramatically decreased in these anemic animals, partially compensated by considerable enhancement in erythropoietic activity. As in humans, these murine sickle cells contain a subpopulation of phosphatidylserine-exposing cells that may play a role in their premature removal. Continuous in vivo generation of this phosphatidylserine-exposing subset may have a significant impact on the pathophysiology of sickle cell disease.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4912-4912
Author(s):  
Marcos André Cavalcanti Bezerra ◽  
Isabela Cristina Farias ◽  
Diego Arruda Falcão ◽  
Igor de Farias Domingos ◽  
Luana Laranjeira Prado ◽  
...  

Abstract Leg ulcers are the most common clinical manifestations of sickle cell anemia (SCA), a monogenic disease with huge clinical diversity among patients. They affect 8% to 10% of SCA patients, reaching a percentage greater than 50% in patients residing in tropical areas. These ulcers occur due to vascular occlusion, tissue hypoxia, hemolysis and genetic factors, presenting a slow healing, high recurrence rate and huge susceptibility to infection. Recently, some studies have shown a positive relationship between the complement system and the development of some vascular diseases and injuries such as leg ulcers in non-SCA patients. Mannan-binding lectin (MBL) is an important component of the humoral innate immune system, and MBL possesses several characteristics indicating that it may play an essential role in wound healing; modulating inflammation and contributing to the clearance of microorganisms and apoptotic cells. In a recent study of chronic leg and foot ulcer patients, serum MBL levels were significantly different between wounds of different etiologies, with chronic venous leg ulcers patients having a higher frequency of MBL deficiency. Polymorphisms in the MBL2 are associated with a reduction in the MBL protein serum levels, increasing risk of developing leg ulcers and also the maintenance of these wounds, compromising the integrity of the immune defence and its response to potential invading pathogens. Here, we aimed to determine the frequency of polymorphisms in the promoter region -221 (Y / X) and -550 (H / L) and exon 1 of the MBL2 and assess the clinical impact of these variants in a northeastern Brazilian SCA population who presented leg ulcers. Two-hundred seventy-five unrelated SCA patients were included. According the leg ulcers presence, the total cohort was classified in patients presenting current or prior history of leg ulcers (n=100) and SCA patients above 18 years with no history of leg ulcers (n=175). Molecular analysis was performed by qPCR. Our population was in Hardy-Weinberg equilibrium. The allelic frequency of haplotypes associated with high MBL production (HYA, LYA) was 54.5% for cases and 62.9% in controls. The genotypes related to low MBL production (HYO, LYO) in cases and controls was 27.5% and 18.6%, respectively. The frequency of genotype related to intermediate MBL production (LXA) was 18% in cases and 18.5% in controls. We had no statistically significant results when we analyzed only the polymorphisms (P>0.05). However, the phenotypic analysis between high and low MBL production revealed that patients with leg ulcers have lower MBL protein levels (P=0.019). We focused specifically on a possible role of MBL deficiency on healing complications, based on the facts that MBL deficiency is the most common immune disorder, and that a common causality for prolonged healing of these ulcers is infection or colonization by bacteria. In our study, MBL deficiency appears to increase the risk of developing leg ulcers in SCA patients. Disclosures No relevant conflicts of interest to declare.


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