108 Platelet Activation in Thalassemic Children

Menichelli, A; Cianciulli, P; Gaviano, E.; Trua, G; Papa, G; Giulio, Stefano Di; Matteis, W. De; Principle, Domenico Del

doi:10.1203/00006450-199112000-00138

Cited by 2 publications

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“…The other mechanism that causes a tendency to thrombosis is platelet activation. Menichelli et al [14] demonstrated in vivo platelet activation in children with TM. In the study of Koçak et al [15] high platelet count found in children with TM increased thrombosis risk.…”

Section: Discussionmentioning

confidence: 99%

Investigation of SCUBE-1 levels in pediatric patients with beta-thalassemia

Acıpayam¹,

Tuncel²,

Güneş³

et al. 2019

Journal of Surgery and Medicine

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Aim: A thrombosis and inflammation marker, Signal peptide-CUB (complement C1r/C1 s)-EGF (epidermal growth factor)-like domaincontaining protein 1 (SCUBE-1) is expressed in endothelial cells and activated platelets. We aimed to investigate the levels of this marker in patients with Thalassemia major (TM), a disease which causes a tendency to thrombosis. Methods: Twenty patients with TM and age and gender-matched twenty healthy controls were included in this prospective case-control study. Other thrombosis markers such as D-dimer and homocysteine were studied in addition to SCUBE-1 in both groups. Results: The SCUBE1 and D-dimer levels were higher in the TM group compared to the control group (P=0.004, P=0.023, respectively). A significant positive correlation was detected between platelet count and SCUBE1 (r=0.342, P=0.031). Conclusion: We detected elevated SCUBE1 levels in children with TM compared to the healthy controls, which correlated with platelet count. SCUBE1 may be used as a thrombosis marker in TM in the early stages of the disease.

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Section: Discussionmentioning

confidence: 99%

Investigation of SCUBE-1 levels in pediatric patients with beta-thalassemia

Acıpayam¹,

Tuncel²,

Güneş³

et al. 2019

Journal of Surgery and Medicine

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Thromboembolic Events in Beta Thalassemia Major: An Italian Multicenter Study

et al. 1998

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Thromboembolic (TE) events have been frequently reported in β-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.

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108 Platelet Activation in Thalassemic Children

Cited by 2 publications

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Investigation of SCUBE-1 levels in pediatric patients with beta-thalassemia

Investigation of SCUBE-1 levels in pediatric patients with beta-thalassemia

Thromboembolic Events in Beta Thalassemia Major: An Italian Multicenter Study

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