Constance P Michel, Laurent A Messonnier, Benoit Giannesini, Christophe Vilmen, Joevin Sourdon, Yann Le Fur, David Bendahan
Sickle cell disease (SCD) is an hemoglobinopathy resulting in the production of an abnormal Hb (HbS) which can polymerize in deoxygenated conditions, leading to the sickling of red blood cells (RBC). These alterations can decrease the oxygen-carrying capacity leading to impaired function and energetics of skeletal muscle. Any strategy which could reverse the corresponding defects could be of interest. In SCD, endurance training is known to improve multiples muscle properties which restores patient's exercise capacity but present reduced effects in anemic patients...
March 29, 2024: Blood Cells, Molecules & Diseases
Karolina Maciak, Aneta Jurkiewicz, Wojciech Strojny, Anna Adamowicz-Salach, Magdalena Romiszewska, Teresa Jackowska, Kinga Kwiecinska, Jaroslaw Poznanski, Monika Gora, Beata Burzynska
Pyruvate kinase (PK) deficiency is a rare autosomal recessive disorder characterized by chronic hemolytic anemia of variable severity. Nine Polish patients with severe hemolytic anemia but normal PK activity were found to carry mutations in the PKLR gene encoding PK, five already known ones and one novel (c.178C > T). We characterized two of the known variants by molecular modeling (c.1058delAAG) and minigene splicing analysis (c.101-1G > A). The former gives a partially destabilized PK tetramer, likely of suboptimal activity, and the c...
March 26, 2024: Blood Cells, Molecules & Diseases
Ivica Marić, Klemen Žiberna, Ana Kolenc, Elvira Maličev
UNLABELLED: Extracellular vesicles (EVs) as membrane-bound particles released by various cells are potential tools for diagnosis and treatment. Blood cells, particularly platelets, are the source of circulating EVs. MATERIAL: EVs were enriched with gradient ultracentrifugation and measured by nanoparticle tracking assay. A flow cytometric multiplex assay was used for cellular source determination. Activation of platelets was measured as a percentage of CD62p+/CD61+ platelets and correlated with the concentration and size of released EVs...
March 13, 2024: Blood Cells, Molecules & Diseases
Sutithi Dey, Rajen Haldar
No abstract text is available yet for this article.
February 29, 2024: Blood Cells, Molecules & Diseases
Shuwei Fan, Guomei Shi, Kelan Li
No abstract text is available yet for this article.
February 28, 2024: Blood Cells, Molecules & Diseases
L Da Costa, Narla Mohandas, Ludivine David-NGuyen, Jessica Platon, Isabelle Marie, Marie Françoise O'Donohue, Thierry Leblanc, Pierre-Emmanuel Gleizes
Diamond-Blackfan anemia (DBA) was the first ribosomopathy described in humans. DBA is a congenital hypoplastic anemia, characterized by macrocytic aregenerative anemia, manifesting by differentiation blockage between the BFU-e/CFU-e developmental erythroid progenitor stages. In 50 % of the DBA cases, various malformations are noted. Strikingly, for a hematological disease with a relative erythroid tropism, DBA is due to ribosomal haploinsufficiency in 24 different ribosomal protein (RP) genes. A few other genes have been described in DBA-like disorders, but they do not fit into the classical DBA phenotype (Sankaran et al...
February 17, 2024: Blood Cells, Molecules & Diseases
Daniel Martínez-Carballeira, Alberto Caro, Ángel Bernardo, José Ramón Corte, José Carlos Iglesias, Isabel Asunción Hernández de Castro, Laura Gutiérrez, Inmaculada Soto
INTRODUCTION: Due to their low prevalence, rare bleeding disorders (RBDs) remain poorly characterized. AIM: To gain insight of RBDs through our clinical practice. METHODS: Retrospective study of the medical records of RBD patients followed up at the Central University Hospital of Asturias between January 2019 and December 2022. RESULTS: A total of 149 patients were included. Factor (F) VII (44 %) and FXI (40 %) deficiencies were the most common diagnosed coagulopathies...
February 15, 2024: Blood Cells, Molecules & Diseases
Auria Godard, Robert Seute, Alexandra Grimaldi, Thomas Granier, Jacques Chiaroni, Wassim El Nemer, Maria De Grandis
BACKGROUND: Erythropoiesis is a complex developmental process in which a hematopoietic stem cell undergoes serial divisions and differentiates through well-defined stages to give rise to red blood cells. Over the last decades, several protocols have been developed to perform ex vivo erythroid differentiation, allowing investigation into erythropoiesis and red cell production in health and disease. RESULTS: In the current study, we compared the two commonly used protocols by assessing the differentiation kinetics, synchronisation, and cellular yield, using molecular and cellular approaches...
January 23, 2024: Blood Cells, Molecules & Diseases
Paul Muteb Boma, Stéphanie Luntadila Ngimbi, Junior Makiese Kindundu, Jean Israël Wela, Nathalie Lukanke Ngoie, Valentin Mukeba Ngwamah, Sandra Mbuyi Tshiswaka, Joséphine Kalenga Monga, Jules Mulefu Panda, Bruno Bonnechère
Sickle cell disease (SCD) is a significant health burden in the Democratic Republic of the Congo (DRC). This study aims to identify predictive factors of mortality in SCD children admitted to emergency care in Lubumbashi, DRC. We performed a non-interventional cohort follow-up on SCD patients aged 0 to 16 admitted for a "true emergency". Demographic, clinical, and biological data were collected. Univariate and multivariate logistic regression analyses were performed to identify significant risk factors associated with mortality...
January 20, 2024: Blood Cells, Molecules & Diseases
Fernanda D'Avila da Silva, Maria Eduarda de Andrade Galiciolli, Ana Carolina Irioda, Cláudia Sirlene Oliveira, Bruna Candia Piccoli, Alessandro de Souza Prestes, Bruna Cogo Borin, Andre Passaglia Schuch, Estael Ochoa-Rodríguez, Yanier Nuñez-Figueredo, João Batista Teixeira da Rocha
JM-20 is a 1,5-benzodiazepine compound fused to a dihydropyridine fraction with different pharmacological properties. However, its potential toxic effects on blood cells have not yet been reported. Thus, the present study aimed to investigate, for the first time, the possible cytotoxicity of JM-20 through cell viability, cell cycle, morphology changes, reactive species (RS) to DCFH-DA, and lipid peroxidation in human leukocytes, its hemolytic effect on human erythrocytes, and its potential DNA genotoxicity using plasmid DNA in vitro...
January 19, 2024: Blood Cells, Molecules & Diseases
Avital Mendelson, Yunfeng Liu, Weili Bao, Patricia A Shi
In preparation for hematopoietic stem cell mobilization and collection, current ex vivo gene therapy protocols for sickle cell disease require patients to undergo several months of chronic red cell transfusion. For health care equity, alternatives to red cell transfusion should be available. We examined whether treatment with GBT1118, the murine analog of voxelotor, could be a safe and feasible alternative to red cell transfusion. We found that 3 weeks of treatment with GBT1118 increased the percentage of bone marrow hematopoietic stem cells and upon plerixafor mobilization, the percentage of peripheral blood hematopoietic stem cells...
January 19, 2024: Blood Cells, Molecules & Diseases
Sangsang Wang, Diao Yu, Junwu Zhang
No abstract text is available yet for this article.
January 17, 2024: Blood Cells, Molecules & Diseases
Lucy Z Kornblith, Bindhya Sadhanandhan, Sreepriya Arun, Rebecca Long, Alicia J Johnson, Jamie Noll, C N Ramchand, John K Olynyk, David H Farrell
No abstract text is available yet for this article.
January 12, 2024: Blood Cells, Molecules & Diseases
Usman Ali Shams, Isma Javed, Muhammad Fizan, Aqib Raza Shah, Ghulam Mustafa, Muhammad Zubair, Yehia Massoud, Muhammad Qasim Mehmood, Muhammad Asif Naveed
Peripheral blood smear examination is one of the basic steps in the evaluation of different blood cells. It is a confirmatory step after an automated complete blood count analysis. Manual microscopy is time-consuming and requires professional laboratory expertise. Therefore, the turn-around time for peripheral smear in a health care center is approximately 3-4 hours. To avoid the traditional method of manual counting under the microscope a computerized automation of peripheral blood smear examination has been adopted, which is a challenging task in medical diagnostics...
January 4, 2024: Blood Cells, Molecules & Diseases
Brígida Santos, Catarina Ginete, Elisângela Gonçalves, Mariana Delgadinho, Armandina Miranda, Paula Faustino, Ana Paula Arez, Miguel Brito
BACKGROUND: Sickle Cell Anemia (SCA) is a monogenic disease, although its severity and response to treatment are very heterogeneous. OBJECTIVES: This study aims to characterize a cohort of Angolan children with SCA and evaluate their response to hydroxyurea (HU) treatment and the potential side effects and toxicity. METHODS: The study enrolled 215 patients between 3 and 12 years old before and after the administration of HU, at a fix dose of 20 mg/kg/day for 12 months...
January 4, 2024: Blood Cells, Molecules & Diseases
Sutithi Dey, Ipsita Chakraborty, Payel Biswas, Ayantika Paul, Pratip Chakraborty, Rajen Haldar
Dyslipidemia is frequently observed in polycystic ovarian syndrome (PCOS). Changes in plasma lipid levels potentially alter erythrocyte membrane lipid composition due to lack of inbuilt lipid synthesis machinery. Therefore, development of morphologically altered erythrocytes in PCOS patients with dyslipidemia is expected. However, this has not been established so far. So, we took this opportunity to explore the morphological alterations among dyslipidemic PCO women. We recruited thirty-five dyslipidemic PCOS women (satisfying Rotterdam criteria, without medication) and twenty-five age-matched healthy controls...
January 3, 2024: Blood Cells, Molecules & Diseases
Yumiko Maruyama, Hidekazu Nishikii, Naoki Kurita, Tatsuhiro Sakamoto, Keiichiro Hattori, Yasuhito Suehara, Yasuhisa Yokoyama, Takayasu Kato, Naoshi Obara, Mamiko Sakata-Yanagimoto, Shigeru Chiba
BACKGROUND: Haploidentical peripheral blood stem cell transplantation (haplo-PBSCT) with post-transplant cyclophosphamide (PTCy) is an important therapeutic option for patients lacking an HLA-matched donor. However, the significance of CD34+ cell dose in grafts has not been fully elucidated. OBJECTIVE: We aimed to explore the impact of CD34+ cell dose on outcomes after haplo-PBSCT with PTCy. STUDY DESIGN: We retrospectively investigated 111 consecutive patients who underwent haplo-PBSCT with PTCy or HLA-matched PBSCT from related donors...
December 30, 2023: Blood Cells, Molecules & Diseases
Marshall A Lichtman, Josef T Prchal
No abstract text is available yet for this article.
December 7, 2023: Blood Cells, Molecules & Diseases
Marshall A Lichtman
Several syndromes affecting the red cell that mimic those induced by germline mutations may result from a somatic mutation that accompanies a myeloid malignancy. These syndromes are most notable in cases of myelodysplastic syndrome, but they are not limited to any one category of myeloid neoplasm. Their occurrence in males exceed the male predominance that is evident in myeloid neoplasms. The syndromes include disorders of globin chain synthesis (α- and β-thalassemia), heme synthesis (erythropoietic porphyria and erythropoietic uroporphyria), red cell membrane structure (elliptocytosis and spherocytosis), red cell enzyme activity (pyruvate kinase deficiency, glucose-6-phosphate dehydrogenase deficiency) and lowered expression of red cell ABO blood group antigens...
November 5, 2023: Blood Cells, Molecules & Diseases
Luis E F Almeida, Meghann L Smith, Sayuri Kamimura, Sebastian Vogel, Zenaide M N Quezado
Red blood cells (RBC) from patients with sickle cell disease (SCD) have elevated calcium levels at baseline, which are further elevated upon deoxygenation. Here we examined baseline calcium levels and calcium flux in RBCs from a mouse model of SCD mice. We found that akin to humans with SCD, sickle (HbSS) Townes mice, have higher baseline levels and increased calcium flux in RBCs compared to control (HbAA) animals. As HbSS mice, unlike humans with SCD, have high mean corpuscular volume compared with HbAA, we highlight the importance of adjusting biochemical results to number of RBCs rather than hematocrit during the analysis and interpretation of the results...
November 2, 2023: Blood Cells, Molecules & Diseases
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