Thromboembolic complications in β-thalassemia: Beyond the horizon (original) (raw)
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Thromboembolism in Beta-Thalassemia Disease
Beta Thalassemia [Working Title]
Thalassemia disease is a common inherited hemolytic anemia frequently found in several parts of the world, especially in the Mediterranean and some Asian countries. Besides the complications of secondary hemochromatosis from regular red blood cell (RBC) transfusion and increased gastrointestinal absorption of iron, thromboembolism (TE) is one of the common long-term complications of beta-thalassemia disease, particularly in patients with non-transfusion-dependent thalassemia (NTDT), which is commonly seen after the second decade of life. The risk factors of TE in beta-thalassemia disease including exposure of phosphatidylserine of abnormal RBCs, increase of platelet activation and aggregation, elevation of endothelial microparticles and increased endothelial activation, decreased nitric oxide (NO) secondary to hemolysis, rise of platelet count and nucleated RBCs after splenectomy, organ dysfunction caused by hemochromatosis, and thrombophilia such as natural anticoagulant deficiencies leading to hypercoagulable state. The understanding of the pathophysiology would result in effective prevention of this complication of beta-thalassemia disease.
Thalassemia and Venous Thromboembolism
Mediterranean Journal of Hematology and Infectious Diseases, 2011
Although the life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in thalassemia identification of a hypercoagulable state in these patients. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemia. Clinical experience and available clues on optimal management are also discussed.
Thalassemia and hypercoagulability
Blood Reviews, 2008
Thalassemia is a congenital hemolytic disease caused by defective globin synthesis resulting in decreased quantity of globin chains. Although the life expectancy of b-thalassemia patients has markedly improved over the last few years, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in b-thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this paper, we review the molecular and cellular mechanisms leading to hypercoagulability in b-thalassemia, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemias. We also discuss the recommendations for thrombosis prophylaxis in these patients.
Pakistan BioMedical Journal
Life expectancy in thalassemia has markedly improved due to consistent blood transfer and amenability with iron chelation therapy, therefore this improvement is conditioned with various thromboembolic problems of this prolonged disorder including thromboembolic complaints. Objective: To determine coagulation abnormalities in beta (β) thalassemia major patients who have been multi transfused. Methods: This observational study was conducted at Department of Haematology & Transfusion Medicine, Children hospital & University of Child Health Sciences (CH&UCHS), Lahore, from October 2022 to January 2023. The study included 60 β-thalassemic patient, age less than 16 years whose samples were compared with upper and lower normal value as regards to Prothrombin Time (PT), Active Partial Thromboplastin Time (APTT), Protein S, Protein C, liver enzymes (Alanine Transaminase (ALT), Aspartate Transaminase (AST), Gamma-Glutamyl Transferase (GGT). The data were analyzed using IBM SPSS version 23.0....
β -Thalassemia: A Current Overview
International Journal of Health Sciences and Research, 2016
Beta-thalassemia is a group of hereditary blood disorders characterized by reduced or absent beta globin chain synthesis, resulting in chronic hemolytic anemia and ineffective erythropoiesis. Individuals with β-thalassemia have widely variable clinical manifestations, extending from nearly asymptomatic to severe anemia requiring lifelong regular blood transfusions and complicated by multiple organ damage. Chronic transfusion inevitably leads to iron overload which necessitates iron chelation therapy. Current overview about the disease clinical picture, method of diagnosis, complications and principles of management is going to be discussed in brief.
Hemostatic parameters in Thalassemia patients; a single institute experience
Journal of the Faculty of Medicine Baghdad, 2016
Background: Changes of coagulation profile have been described in patients with B-Thalassemia major. Prolongation of prothrombin time and partial thromboplastin time and reduced levels of coagulation factors and natural anticoagulants have been variously described though the mechanisms involved in the thrombotic tendency seen in some Thalassemia patients have not been fully clarified. Objectives: To investigate changes in the coagulation profile in patients with B-Thalassemia according to the available hemostatic parameters in Thalassemia patients in Al-Karama Teaching Hospital in Wasit governorate. Methods: Hemostatic variables were studied in pre-transfusion blood samples from 50 transfusion-dependent children with Beta Thalassemia (mean age 13.2 years) and from 20 healthy controls. Results: Laboratory evaluation showed thrombocytopenia in 43.5%, prolongation of prothrombin time (PT) in 54% and prolongation of activated partial thromboplastin time (aPTT) in 56% of the patients. Al...
Common Complications in Beta-Thalassemia Patients
International Journal of Hematology and Oncology, 2013
Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 ß-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with diagnosis of thalassemia major and 11 with thalassemia intermedia. Totally, 56.7% of patients were male and 43.3% were female. Ages varied between 2-20 years with the mean age of 10.3±4.8 years. Mean ferritin level was 2212±1370 ng/mL (41-6263 ng/mL) for 4.5 years. Complications were increased with increasing age. Complication rates were significantly higher among thalassemia major patients compared to thalassemia intermedia patients. There was no statistically significant relationship between complications and mean ferritin levels. The most common complications were endocrine complications (38.8%). Cardiac complications developed in 22.4% of the patients; gastroenterological complications in 19.4%; allergic complications in 9%; infectious complications in 1.5%; and thrombosis was detected in 1.5%. The endocrine complications were osteoporosis, growth retardation, developmental delay, short stature, hypothyroidism, delayed puberty, hypogonadism, and diabetes mellitus. The cardiac complications were left ventricular wall hypertrophy, diastolic dysfunction, systolic dysfunction, heart failure, pericardial effusion, dilated cardiomyopathy, left ventricular dilatation, left atrial dilatation, and fatal arrhythmias. Mortality occurred in one (1.5%) out of 67 ß-thalassemia patient due to dilated cardiomyopathy and fatal arrhythmia.
Subclinical haemorrhagic tendency exists in patients with β-thalassaemia major in early childhood
The Australasian medical journal, 2012
Alterations of coagulation profile have been reported in patients with β-thalassaemia major (β-TM). To investigate this in the paediatric population, we studied haemostatic parameters in pre-transfusion blood samples from 50 non-splenectomised transfusion-dependent children with β-TM (mean age 6±2.5 years) and in blood from 25 healthy controls. Laboratory evaluation showed thrombocytopenia in 40%, prolongation of prothrombin time (PT) in 12% and prolongation of activated partial thromboplastin time (APTT) in 6% of the patients. Mean values for PT, APTT and platelet count (PC) were all raised in the patient population compared with the controls. The alteration of coagulation status was significant for PT (p value <0.005) and APTT (p value <0.0001). However, the change for PC was not significant (p value <0.05). No significant liner correlation could be identified between PT, APTT, PC of the patients and interval between transfusions (in days) or days since last transfusion. ...