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PedAM

Pediatric Disease Annotations & Medicines




Disease thalassemia
Phenotype C0398623|hypercoagulable state
Sentences 3
PubMedID- 25286743 thalassaemia is associated with a hypercoagulable state caused by aggregation of platelets and red-blood-cells (rbcs), increased thrombin production and vasoconstriction 53. as a result, patients are at higher risk of thromboembolic events, and this risk may be up to fourfold higher in patients with β-ti compared with regularly transfused patients with β-thalassaemia major 44. in 584 patients with β-ti enrolled in the optimal care study, splenectomy, older age (over 35 years) and high serum ferritin (≥1000 μg/l) were significantly associated with increased risk of thrombosis, whereas transfusion therapy significantly decreased the risk by more than 70% 23. patients with β-ti are also at increased risk of silent brain infarcts, with one study finding that 18 out of 30 splenectomized patients had evidence of asymptomatic white matter lesions by magnetic resonance imaging (mri) 54. older patients and those not previously transfused had the highest incidence and number of lesions.
PubMedID- 20948876 Finally, the hypercoagulable state associated with scd and thalassemia has been shown to contribute to the development of ph through a series of recent experiments.
PubMedID- 23984004 One of these is a hypercoagulable state, particularly in patients with thalassemia intermedia (1, 2).

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