The mechanism of the well observed hypercoagulability and high incidence of Thromboembolic Events (TE) in β- thalassemia patients has not been fully elucidated. This study aimed to evaluate evaluate the endothelial dysfunction and monocyte activation among adult Egyptian β-thalassemic patients and assess their role in the hypercoagulability and development of TE. A total of 40 adults patients with bthalassemics and 20 healthy age and sex-matched controls were assessed for endothelial dysfunction using serum Von Willebrand Factor Antigen (VWFAg) and for monocytic activation using flow cytometric assessment of CD14 monocyte microparticles and CD11b activated monocytes. The VWF:Ag level was significantly higher among thalassemic patients (P<0.001) and was positively correlated to development of TE (P<0.05). There was no significance difference for CD14 between patients and controls (P>0.5) and CD11b was higher in controls (P=0.004) with no significant correlation between both and TE development (P>0.05). VWF:Ag is increased in thalassemic patients and could be used as a risk factor for thrombosis in these patients, while no identified role of activated monocytes in thrombotic tendency in such patients.

Fetal hemoglobin (HbF) is a potent genetic modifier of β-thalassemia phenotype. B-cell lymphoma 11A (BCL11A) gene results in significant silencing of HbF. The aim of this study was to assess the prevalence of different BCL11A genotypes among a cohort of Egyptian children with β-thalassemia and to correlate them to HbF and clinical severity score. Eighty-two children with β-thalassemia (aged 12.95 ± 3.63 years) were recruited from the Pediatric Hematology Clinic, Ain Shams University. They were divided based on the clinical severity of β-thalassemia into three subgroups: 20 mild (24.4%), 24 moderate (29.3%), and 38 severe (46.3%). Age, gender, age of diagnosis, initial HbF level, transfusion history, and history of splenectomy were assessed. Anthropometric measures, signs of anemia and hemosiderosis, and the severity score were determined. Laboratory investigations such as complete blood picture, ferritin, and single gene polymorphism genotyping of the rs11886868 were also performed. Our findings showed that 16 children had CC genotype (19.5%), 38 had TC genotype (46.3%), and 28 had TT genotype (34.1%) of the rs#. β-thalassemia children with TT genotype had significantly higher severity scoring than the other two groups (p < 0.001). Moreover, mean initial HbF was found to be lower in children with TT genotype followed by TC and CC genotypes (p < 0.001). Increased γ-globin expression associated with BCL11A gene polymorphism is associated with better clinical severity of β-thalassemia.

Background: CD47 is an integrin associated protein which decreases with PRBCs (packed red blood cells) prolonged storage that possibly leads to less effective blood transfusion. Electrolyte imbalance occurs due to inactivation of Na+/K+ ATPase pump in low temperature which in turn leads to continuous increase of extracellular potassium, which is considered life threatening for patients with renal failure or those with massive transfusions. The objective of the present study is to evaluate the expression of CD47 by flow cytometry with other biochemical storage changes (pH, sodium and potassium ions) in CPD/SAGM packed non-leucoreduced Group (A) versus CPD/SAGM leucoreduced Group (B) red blood cell units at different storage times (on day 7, 14, 28 from date of red blood cells collection).Subjects and Methods: Nearly 450 ml of whole blood is obtained into quadruple blood bags and further subdivided into 20 non-leucoreduced units and 20 leucoreduced units. Non-leucoreduced and leucoreduced units are stored at 4 ± 2•C. Samples are taken from each packed red blood cell unit at days 7, 14 and 28 and tested for Na+ and K+ ions, pH and CD47 marker by flow cytometry.Results: Both leucoreduced and non-leucoreduced units show a highly significant decrease in Na+, pH and CD47 expression and a highly significant increase in K+ level. However, there was no statistically significant difference found between the 2 studied groups regarding Na+ and K+ levels, pH and CD47 expression on packed red cells except for Na+ on 28 th day which was slightly higher in leucoredced than non-leucoreduced units. Conclusion:Red cell storage lesion is an inevitable process. Our study showed a highly statistically significant decrease in CD47 by flow cytometry in both leucoreduced and non-leucoreduced PRBCs along the storage period from day 7 to day 28.

Background Programed cell death protein 1 (PD‐1) is a key mediator for the development of T cell exhaustion that develops in response to persistent antigen stimulation. Aim In this study, we measured PD1 expression on CD3 positive bone marrow T‐lymphocytes in newly diagnosis AML patients and its relation to clinical/ prognostic outcomes in addition to response to induction therapy (day 28). Methods This study was conducted on 59 newly diagnosed AML patients and 20 healthy controls. Complete blood counts, flow cytometry using acute leukemia panel in addition to PD1 monoclonal antibodies were performed on bone marrow lymphocytes (CD3+), whereas cytogenetic/molecular studies were used to determine risk group. The patients’ remission status following induction therapy was determined. Results PD1 was brightly expressed in 91.5% of the cases than control sample with highly significant difference (P = .001). A cutoff of 3.5 for mean fluorescence intensity was used to divide patients into two groups (higher vs normal PD1 expression). A significant difference between the two groups regarding platelet count and aberrant CD7 expression (P = .007 and .023, respectively) was found. Those normally expressed PD1 respond better to induction therapy. Conclusion PD1 expression on BM T‐cells had a predictive value and providing an immunotherapeutic target for AML.

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