Study of Platelet Activation, Hypercoagulable State, and the Association with Pulmonary Hypertension in Children with β-Thalassemia

Publication date: Available online 15 June 2017
Source:Hematology/Oncology and Stem Cell Therapy
Author(s): Mahmoud Alhosiny Fayed, Hesham El-Sayed Abdel-Hady, Mona Mohammed Hafez, Osama Saad Salama, Youssef Abdelhalim Al-Tonbary
BackgroundThe increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered.ObjectiveStudy 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.Methods36 Egyptian children with β-thalassemia with a mean age of 9.9 years (±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] >25 mmHg.ResultsWe found that, mean ±SD serum P.selectin level (platelet activator marker) was significantly higher in thalassemic patients (2337 ±566 pg/ml) in comparison to controls (1467 ±247 pg/ml) (P<0.001).Mean serum protein-C and antithrombin-III levels were significantly lower in thalassemic patients (1.2±1.3 µg/ml, 27.3 ±7.5 mg/dl) in comparison to controls (2.3 ±1.3 µg/ml, 35.1 ±4.1 mg/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).ConclusionA 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.



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