scholarly journals Peripheral Red Blood Cell Split Chimerism as a Consequence of Intramedullary Selective Apoptosis of Recipient Red Blood Cells in a Case of Sickle Cell Disease

2014 ◽  
Vol 6 (1) ◽  
pp. e2014066 ◽  
Author(s):  
Marco Marziali ◽  
Antonella Isgrò ◽  
Pietro Sodani ◽  
Javid Gaziev ◽  
Daniela Fraboni ◽  
...  

Allogeneic cellular gene therapy through hematopoietic stem cell transplantation is the only radical cure for congenital hemoglobinopathies like thalassemia and sickle cell anemia. Persistent mixed hematopoietic chimerism (PMC) has been described in thalassemia and sickle cell anemia. Here, we describe the clinical course of a 6-year-old girl who had received bone marrow transplant for sickle cell anemia. After the transplant, the patient showed 36% donor hematopoietic stem cells in the bone marrow, whereas in the peripheral blood there was evidence of 80%  circulating donor red blood cells (RBC). The analysis of apoptosis at the Bone Marrow  level suggests that Fas might contribute to the cell death of host erythroid precursors. The increase in NK cells and the regulatory T cell population observed in this patient suggests that these cells might contribute to the condition of mixed chimerism.

Blood ◽  
2001 ◽  
Vol 97 (12) ◽  
pp. 3960-3965 ◽  
Author(s):  
Robert Iannone ◽  
Leo Luznik ◽  
Laura W. Engstrom ◽  
Sherrie L. Tennessee ◽  
Frederic B. Askin ◽  
...  

Sickle cell anemia (SCA) is an inherited disorder of β-globin, resulting in red blood cell rigidity, anemia, painful crises, organ infarctions, and reduced life expectancy. Allogeneic blood or marrow transplantation (BMT) can cure SCA but is associated with an 8% to 10% mortality rate, primarily from complications of marrow-ablative conditioning. Transplantation of allogeneic marrow after less intensive conditioning reduces toxicity but may result in stable mixed hematopoietic chimerism. The few SCA patients who inadvertently developed mixed chimerism after BMT remain symptom free, suggesting that mixed chimerism can reduce disease-related morbidity. However, because the effects of various levels of mixed chimerism on organ pathology have not been characterized, this study examined the histologic effects of an increasing percentage of normal donor hematopoiesis in a mouse model of BMT for SCA. In lethally irradiated normal mice that were reconstituted with varying ratios of T-cell–depleted marrow from normal and transgenic “sickle cell” mice, normal myeloid chimerism in excess of 25% was associated with more than 90% normal hemoglobin (Hb). However, 70% normal myeloid chimerism was required to reverse the anemia. Organ pathology, including liver infarction, was present in mice with sickle Hb (HbS) levels as low as 16.8% (19.6% normal myeloid chimerism). Histologic abnormalities increased in severity up to 80% HbS, but were less severe in mice with more than 80% HbS than in those with 40% to 80% HbS. Therefore, stable mixed chimerism resulting from nonmyeloablative BMT may reduce the morbidity from SCA, but prevention of all disease complications may require minimizing the fraction of circulating sickle red cells.


2021 ◽  
Vol 26 (09) ◽  
Author(s):  
Endris Muhammed ◽  
James Cooper ◽  
Daniel Devito ◽  
Robert Mushi ◽  
Maria del Pilar Aguinaga ◽  
...  

2019 ◽  
Vol 7 (6) ◽  
pp. e14027 ◽  
Author(s):  
Halima Al Balushi ◽  
Kobina Dufu ◽  
David C. Rees ◽  
John N. Brewin ◽  
Anke Hannemann ◽  
...  

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.


Haematologica ◽  
2016 ◽  
Vol 101 (12) ◽  
pp. e469-e472 ◽  
Author(s):  
S. Tewari ◽  
D. C. Rees ◽  
A. Hannemann ◽  
O. T. Gbotosho ◽  
H. W. M. Al Balushi ◽  
...  

2017 ◽  
Vol 50 ◽  
pp. 34-41 ◽  
Author(s):  
Xuejin Li ◽  
Ming Dao ◽  
George Lykotrafitis ◽  
George Em Karniadakis

2010 ◽  
Vol 13 (1) ◽  
pp. 1-11 ◽  
Author(s):  
Chun-Seok Cho ◽  
Gregory J. Kato ◽  
Seung Ha Yang ◽  
Sung Won Bae ◽  
Jong Seo Lee ◽  
...  

2000 ◽  
Vol 65 (2) ◽  
pp. 174-175 ◽  
Author(s):  
Maxwell P. Westerman ◽  
Yin Zhang ◽  
Joseph P. McConnell ◽  
Paul A. Chezick ◽  
Rakshanda Neelam ◽  
...  

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.


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