Background And Objectives: The aim of this study was to investigate the T helper (Th) to T cytotoxic (Tc) ratio in children suffering from type A hemophilia disease and to evaluate the correlation of this ratio with disease severity.
Material And Method: Two mls of EDTA anti coagulated whole blood was collected. Immunophenotyping of lymphocytes count was carried out by FACS analysis using a double CD4 and CD8 kit. The mean ± SD of absolute numbers of CD4 and CD8 lymphocytes/ml was calculated and the ratio of CD4/CD8 was evaluated by statistical method.
Results: Among 80 type A hemophilia patients, 66 (82.5%) were male. The mean age was 15 ± 3.51 years. 12 (15%) of them were suffering from mild disease and 68 (85%) had sever disease. The CD4 /CD8 ratio was obtained between 0.45 and 1.44 with mean1.79 ± 0.78. The correlation between this ration and disease severity was 0.019.
Conclusion: The results showed that CD4/CD8 ratio has correlation with disease severity in type A hemophilia patients, however there was no association between this ratio and gender.
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http://dx.doi.org/10.3233/HAB-200434 | DOI Listing |
Exp Mol Med
January 2025
Department of Physiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.
Hemophilia A (HA) is caused by mutations in coagulation factor VIII (FVIII). Genome editing in conjunction with patient-derived induced pluripotent stem cells (iPSCs) is a promising cell therapy strategy, as it replaces dysfunctional proteins resulting from genetic mutations with normal proteins. However, the low expression level and short half-life of FVIII still remain significant limiting factors in the efficacy of these approaches in HA.
View Article and Find Full Text PDFIntroduction: Hereditary bleeding disorders stem from the absence or insufficient levels of particular clotting proteins, essential for facilitating coagulation in the clotting cascade. Among the most prevalent are hemophilia A (deficiency of Factor VIII), hemophilia B (deficiency of Factor IX), and von Willebrand disease. Management of pharmacoresistant epilepsy is more difficult in a patient with bleeding disorder due to increased risk of bleeding during surgery.
View Article and Find Full Text PDFTher Adv Hematol
December 2024
Departments of Pediatrics and Pathology, University of Michigan, Ann Arbor, MI, USA.
Background: Hemophilia A is caused by coagulation factor VIII (FVIII) deficiency and increases bleeding risk during invasive procedures.
Objectives: To investigate FVIII concentrate use and bleeding outcomes for invasive procedures after valoctocogene roxaparvovec gene transfer.
Design: This manuscript presents post hoc analysis of the phase III GENEr8-1 trial.
J Allergy Clin Immunol Pract
December 2024
EUCAN Medical Affairs, Takeda Pharmaceuticals International AG, Glattpark-Opfikon (Zürich), Switzerland.
Background: Hereditary angioedema (HAE) is a rare genetic disease characterized by recurrent episodes of cutaneous or subcutaneous edema. There is clinical need for treatments that reduce the rate of HAE attacks in patients.
Objectives: Primary objectives were to evaluate the effectiveness of lanadelumab on attack free rate (AFR; proportion of patients who had zero HAE attacks), and on every two weeks (Q2W) and every four weeks (Q4W) adjustments on AFR.
Haemophilia
December 2024
Institute of Experimental Hematology and Transfusion Medicine, Medical Faculty, University Hospital Bonn, University of Bonn, Bonn, Germany.
Introduction: Maintaining the balance between procoagulant and anticoagulant factors is essential for effective haemostasis. Emerging evidence suggests a modulation of bleeding tendency by factors in the anticoagulant and fibrinolytic systems.
Aim: This study investigates the clinical and laboratory characteristics of a family with combined von Willebrand disease (VWD) and antithrombin (AT) deficiency.
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