Mahmoud Alhosiny Fayed1, Hesham El-Sayed Abdel-Hady2, Mona Mohammed Hafez3, Osama Saad Salama4, Youssef Abdelhalim Al-Tonbary5. 1. Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt. Electronic address: Drmahmoud38@hotmail.com. 2. Neonatal Intensive Care Unit, Pediatric Department, Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt. Electronic address: hehady@mans.edu.eg. 3. Cardiology Unit, Pediatric Department, Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt. Electronic address: monammhafez@mans.edu.eg. 4. Hematology Unit, Clinical Pathology Department, Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt. Electronic address: o_salama@mans.edu.eg. 5. Hematology/Oncology Unit, Pediatric Department, Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt. Electronic address: ytonbary@mans.edu.eg.
Abstract
BACKGROUND: The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9.9years (±4.7 SD). In addition, 20 healthy Egyptian children matched for age and sex were enrolled as a control group. Both were subjected to clinical and laboratory assessments. Echocardiography was done to the patient group and PH was diagnosed based on calculated mean pulmonary artery pressure [MPAP] >25mmHg. RESULTS: We found that, mean±SD serum P-selectin level (platelet activator marker) was significantly higher in thalassemic patients (2337±566pg/ml) in comparison to controls (1467±247pg/ml) (P<0.001). Mean serum protein-C and antithrombin-III levels were significantly lower in thalassemic patients (1.2±1.3µg/ml, 27.3±7.5mg/dl) in comparison to controls (2.3±1.3µg/ml, 35.1±4.1mg/dl) (P=0.003 and <0.001) respectively. PH was detected in 17 (47.2%) patients and it was significantly associated with splenectomy (P=0.01) and non-transfusion dependent thalassemia (NTDT) (P=0.04). PH was positively correlated with serum levels of P-selectin (r=0.38, P=0.02), fibrinogen (r=0.41, P=0.01) and negatively correlated with serum protein-C level (r=-0.48, P=0.003). CONCLUSION: A chronic hypercoagulable state and platelet activation is present in children with β-thalassemia. Splenectomy and transfusion infrequency are the main risk factors noted to be associated with such hypercoagulable state and platelet activation and consequently the PH among our thalassemic patients.
BACKGROUND: The increased survival rate of thalassemicpatients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9.9years (±4.7 SD). In addition, 20 healthy Egyptian children matched for age and sex were enrolled as a control group. Both were subjected to clinical and laboratory assessments. Echocardiography was done to the patient group and PH was diagnosed based on calculated mean pulmonary artery pressure [MPAP] >25mmHg. RESULTS: We found that, mean±SD serum P-selectin level (platelet activator marker) was significantly higher in thalassemicpatients (2337±566pg/ml) in comparison to controls (1467±247pg/ml) (P<0.001). Mean serum protein-C and antithrombin-III levels were significantly lower in thalassemicpatients (1.2±1.3µg/ml, 27.3±7.5mg/dl) in comparison to controls (2.3±1.3µg/ml, 35.1±4.1mg/dl) (P=0.003 and <0.001) respectively. PH was detected in 17 (47.2%) patients and it was significantly associated with splenectomy (P=0.01) and non-transfusion dependent thalassemia (NTDT) (P=0.04). PH was positively correlated with serum levels of P-selectin (r=0.38, P=0.02), fibrinogen (r=0.41, P=0.01) and negatively correlated with serum protein-C level (r=-0.48, P=0.003). CONCLUSION: A chronic hypercoagulable state and platelet activation is present in children with β-thalassemia. Splenectomy and transfusion infrequency are the main risk factors noted to be associated with such hypercoagulable state and platelet activation and consequently the PH among our thalassemicpatients.