Objective: To study the effect of splenectomy in patients with thalassemia major on the cardiovascular system through echocardiographic study.
Methods: A prospective, cross sectional study was carried out from December 2006 to December 2007. Patients from the Thalassemia Center in the Maternity and Children's Hospital, Madina, Kingdom of Saudi Arabia, were screened by means of history, physical examination, laboratory studies, and echocardiography.
Results: Fifty-seven patients were studied: 36 were non-splenectomized, while 21 were surgically splenectomized. The 2 study groups were well matched for age, gender, height, and weight. The total amount of blood given during the previous year (6577.1+/-206.9 ml versus 5390.5+/-220.2 ml, p=0.0005), and the annual transfusion index (200.9+/-11.3 versus 134.1+/-7.3, p=0.0001) were significantly lower in the splenectomized group. There was no significant difference between the 2 groups regarding laboratory studies. Left ventricular systolic function shows no difference regarding fraction shortening between the 2 groups. The mitral valve E/A ratio was significantly higher in the splenectomized group (1.6+/-0.2 versus 1.4+/-0.2, p=0.02). The pulmonary artery pressure was higher in the splenectomized group (34.2+/-9.1 versus 20.8+/-9.2 mm Hg, p=0.0001). There was a significantly higher number of patients with pulmonary hypertension in the splenectomized group (14 [66.7%] versus 6 [16.7%], p=0.0004).
Conclusion: Splenectomized patients with thalassemia major are at high risk of having impaired diastolic left ventricular function and pulmonary hypertension.
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Mol Biol Rep
January 2025
Thalassemia & Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Introduction: Hematologic malignancies, originating from uncontrolled growth of hematopoietic and lymphoid tissues, constitute 6.5% of all cancers worldwide. Various risk factors including genetic disorders and single nucleotide polymorphisms play a role in the pathogenesis of hematologic malignancies.
View Article and Find Full Text PDFArch Gynecol Obstet
January 2025
Department of Obstetrics & Gynecology, University of Tabuk, Tabuk, Saudi Arabia.
Purpose: We explored the effect of beta-thalassemia major on pregnancy and delivery outcomes in non-endemic area, utilizing USA population database.
Methods: This is a retrospective study utilizing data from the Healthcare Cost and Utilization Project-Nationwide Inpatient Sample. A cohort of all deliveries between 2011 and 2014 was created using ICD-9 codes.
Zhongguo Dang Dai Er Ke Za Zhi
January 2025
, Department of Pediatrics, Third Xiangya Hospital, Central South University Changsha 410013 China.
To guide clinical blood transfusion practices for pediatric patients, the National Health Commission has issued the health standard "Guideline for pediatric transfusion" (WS/T 795-2022). Blood transfusion is one of the most commonly used supportive treatments for children with hematological diseases. This guideline provides guidance and recommendations for blood transfusions in children with aplastic anemia, thalassemia, autoimmune hemolytic anemia, glucose-6-phosphate dehydrogenase deficiency, acute leukemia, myelodysplastic syndromes, immune thrombocytopenic purpura, and thrombotic thrombocytopenic purpura.
View Article and Find Full Text PDFAnemia
January 2025
Department of Hematology, Mehmet Akif Inan Egitim Ve Arastirma Hastanesi, Sanliurfa, Turkey.
The effect of pretransfusion hemoglobin on transfusion burden, thrombosis, and mortality in thalassemia and myelodysplastic syndrome is unclear. We aimed to study the pretransfusion hemoglobin and erythrocyte transfusion burden and investigate the effects of these variables on each other in real-life in thalassemia and myelodysplastic syndrome. Adult patients with thalassemia and myelodysplastic syndrome who received at least one erythrocyte concentrate unit outpatient at Sanliurfa Mehmet Akif Inan Training and Research Hospital during 1 year were included in the study.
View Article and Find Full Text PDFAsian J Transfus Sci
September 2022
Department of Zoology, CCS HAU, Hisar, Haryana, India.
Context: Hemoglobinopathies are the most common heterogeneous group of monogenetic disorder in the world and its prevalence varies with geographical regions. India is developing country and many studies show a significant burden of hemoglobinopathies in India.
Aims: The aim of the present study was to check the prevalence of various hemoglobinopathies in anemic subjects using high-performance liquid chromatography (HPLC) method in Pune region which has multiple ethnic population groups from all parts of India.
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